heme/onc 6 Flashcards
(468 cards)
1
Q
What is melanoma?
A
Melanoma is a type of skin cancer that develops from the pigment-producing cells known as melanocytes.
2
Q
What is renal-cell carcinoma?
A
Renal-cell carcinoma is a type of kidney cancer that originates in the lining of the renal tubules.
3
Q
What types of cancer are associated with BCR-ABL rearrangement?
A
BCR-ABL rearrangement is common in chronic myeloid leukemia (CML).
4
Q
What rare disease is a serum tryptase level diagnostic of
A
A serum tryptase level >20 ng/mL in the absence of an associated myeloid disorder is a minor diagnostic criterion for systemic mastocytosis.
5
Q
What mutation causes hereditary hemochromatosis (HH)?
A
HH is most commonly caused by mutations in the HFE gene, such as C282Y or H63D, and typically manifests in middle-aged white men.
6
Q
What is secondary hemostasis?
A
Secondary hemostasis is the stabilization of the platelet plug (white thrombus) by creating a fibrin network.
7
Q
What is the most likely diagnosis for a young adult with a destructive bony lesion near the knee?
A
The most likely diagnosis is osteosarcoma, especially with radiographic findings consistent with a sunburst pattern.
8
Q
What is the best initial management for patients with metastatic cancer and intractable pain?
A
The best initial management is a patient-controlled analgesia pump with an intravenous opioid.
9
Q
What drug has been associated with significant improvements in overall survival among patients receiving carboplatin and paclitaxel?
A
Bevacizumab has been associated with significant improvements in overall survival among these patients.
10
Q
What are myeloproliferative neoplasms (MPNs)?
A
MPNs are the most common acquired risk factor for Budd-Chiari syndrome.
11
Q
What effect do diminished levels of von Willebrand factor have?
A
Diminished levels of von Willebrand factor increase clearance of factor VIII and lead to a factor VIII deficiency.
12
Q
What virus is associated with hepatitis C?
A
Hepatitis C virus (HCV) can lead to various complications, including liver disease.
13
Q
What virus is associated with the development of lymphomas?
A
HCV is associated with the development of lymphomas of B-cell origin, particularly splenic lymphoma.
14
Q
What is the most common type of sarcoma in children and adolescents?
A
Ewing sarcoma is most common in children and adolescents.
15
Q
What reagent should be used to assess for pseudothrombocytopenia?
A
To assess for pseudothrombocytopenia, repeat testing with citrate instead of EDTA.
16
Q
What does EDTA cause in patients?
A
EDTA causes the development of platelet autoantibodies resulting in clumping.
17
Q
What is the most appropriate management for cancer pain crisis with opioid-induced neurotoxicity?
A
The most appropriate management is opioid rotation with dose reduction to account for incomplete cross-tolerance.
18
Q
What may beta-thalassemia intermedia require?
A
Beta-thalassemia intermedia may require intermittent blood transfusions.
19
Q
Where are varicoceles fairly common?
A
Varicoceles are fairly common on the left side because of the higher pressure within the left gonadal vein compared with the right gonadal vein.
20
Q
What does beta-thalassemia lead to?
A
Beta-thalassemia leads to abnormal erythrocytes that express procoagulant phospholipids, activated clotting factors, and activated platelets, all of which predispose patients to thrombosis.
21
Q
What is the most appropriate treatment for diarrhea and flushing associated with carcinoid syndrome?
A
The most appropriate treatment is a somatostatin analogue such as octreotide.
22
Q
What does hydroxyurea increase?
A
Hydroxyurea increases levels of hemoglobin F.
23
Q
What is the most likely diagnosis in a patient with pancytopenia without myeloid immaturity?
A
The most likely diagnosis is Gaucher disease.
24
Q
What is the likely diagnosis in an asymptomatic person with a mean corpuscular volume in the 60s but normal hemoglobin?
A
The likely diagnosis is beta-thalassemia trait or alpha-thalassemia trait.
25
What happens to von Willebrand factor levels in certain conditions?
Diminished levels of von Willebrand factor increase.
26
What factor is diminished in von Willebrand disease?
Diminished levels of von Willebrand factor.
27
What does increased clearance of factor VIII lead to?
A deficiency in factor VIII.
28
How does the left gonadal vein drain?
It drains at a right angle into the left renal vein.
29
How does the right gonadal vein insert?
It inserts into the inferior vena cava at a more acute angle.
30
What is the most appropriate management of hypercalcemia of malignancy?
Aggressive volume repletion followed by either an intravenous bisphosphonate or denosumab.
31
What are superwarfarins?
High-potency vitamin K antagonists used as rat poisons.
32
What is the most likely cause of night sweats, weight loss, and cervical lymphadenopathy in a patient with common variable immunodeficiency?
Non-Hodgkin lymphoma.
33
What features are associated with increased risk for progression of MGUS?
A serum monoclonal protein level >1.5 g/dL, non-immunoglobulin G monoclonal protein, and a markedly abnormal free-light-chain-kappa-to-lambda ratio.
34
What is TRALI commonly associated with?
Hypotension and normal/elevated central venous pressure.
35
What is the recommended follow-up for a smoker with a single solid pulmonary nodule 6 to 8 mm in size?
A follow-up low-dose CT scan of the chest in 6 months.
36
What may ITP patients who relapse or are unresponsive to dexamethasone respond to?
Intravenous immunoglobulin or stimulation of platelet production with eltrombopag or romiplostim, or immunosuppression with rituximab or other immunosuppressive agents.
37
What is reserved for patients refractory to medical management in ITP?
Splenectomy.
38
What forms a complex in the extrinsic pathway of coagulation?
Factor VIIa and tissue factor.
39
What is thrombocytosis often seen in?
Beta thalassemia patients due to increased drive on the marrow from erythropoietin.
40
What are dabigatran and rivaroxaban?
Anticoagulants.
41
Which anticoagulants are not recommended in patients with end-stage kidney disease?
Rivaroxaban and apixaban are not recommended in patients with end-stage kidney disease.
42
What is likely in patients with chronic kidney failure?
Functional iron deficiency is considered to be likely in patients with chronic kidney failure.
43
What ferritin level indicates functional iron deficiency in chronic kidney failure?
A ferritin level of ≤500 ng/mL indicates functional iron deficiency in chronic kidney failure.
44
What transferrin saturation level indicates functional iron deficiency?
A transferrin saturation of ≤30% indicates functional iron deficiency.
45
What is the difference in RDW between beta thalassemia and iron deficiency anemia?
In beta thalassemia, RDW is normal; in iron deficiency anemia, RDW is elevated.
46
When are erythropoiesis-stimulating agents started?
Erythropoiesis-stimulating agents are started when the hemoglobin level falls below 10 g/dL.
47
What hemoglobin level is critical to avoid falling below?
The hemoglobin concentration should not fall below 9 g/dL.
48
What are the most common primary tumors that metastasize to the brain?
The most common primary tumors that metastasize to the brain are lung cancer, melanoma, renal-cell carcinoma, and breast cancer.
49
What condition is indicated by plasmacytoid lymphocytes in the bone marrow?
Plasmacytoid lymphocytes in the bone marrow indicate Waldenström macroglobulinemia.
50
What does the treatment of TLS include?
The treatment of TLS includes rasburicase and aggressive volume expansion with normal saline.
51
What is the target urine output for most adults during TLS treatment?
The target urine output is around 150–200 mL/hour for most adults.
52
What is an important cause of chronic diarrhea following partial pancreatectomy?
Exocrine pancreatic insufficiency is an important cause of chronic diarrhea following partial pancreatectomy.
53
What is the best laboratory test to diagnose ACTH-associated paraneoplastic syndrome?
The best laboratory test is an overnight dexamethasone suppression test or a 24-hour urinary free cortisol test.
54
What is checked after administering dexamethasone in the overnight test?
The morning cortisol level is checked after administering 1 mg of dexamethasone at 11 p.m. the night before.
55
What indicates leukocytosis in a patient?
Leukocytosis is indicated by a white blood cell count above 100,000 cells per mm³ with absolute basophilia.
56
What is the most likely diagnosis in a patient with leukocytosis above 100,000 cells per mm3 with absolute basophilia, thrombocytosis, and splenomegaly?
The most likely diagnosis is chronic myeloid leukemia.
57
What is the classic appearance of meningiomas on MRI?
Meningiomas are adjacent to bone and anchored to the dura, rendering the classic appearance of the 'dural tail' on imaging.
58
What condition is characterized by immune-mediated necrotizing vasculitis affecting small and medium-sized vessels in the upper and lower respiratory tract, eyes, and kidneys?
Likely diagnosis: Granulomatosis with polyangiitis.
59
What is the diagnosis associated with a thrombotic event occurring in the context of persistently positive lupus anticoagulant or anticardiolipin antibody?
Diagnosis: Antiphospholipid syndrome.
60
What is the characteristic development timeline for therapy-related acute myeloid leukemia?
Classically develops 5 to 7 years after treatment with alkylating chemotherapy agents and radiotherapy.
61
What is the most likely diagnosis in a young adult with a destructive bony lesion near the knee and radiographic findings consistent with a sunburst pattern?
The most likely diagnosis is osteosarcoma.
62
What is the risk category for patients with acute lymphocytic leukemia and a leukocyte count >50,000 cells per mm3?
They are in the highest risk category for tumor lysis syndrome.
63
What is Bevacizumab?
Bevacizumab is a monoclonal antibody against vascular endothelial growth factor.
64
What is the characteristic erythrocyte morphology in patients with beta-thalassemia?
Patients have uniformly microcytic erythrocytes.
65
What is the most likely diagnosis in a patient with splenomegaly and teardrop-shaped erythrocytes, nucleated erythrocytes, and immature granulocytes?
The most likely diagnosis is primary myelofibrosis.
66
What is primary myelofibrosis?
Primary myelofibrosis is a type of blood cancer that affects the bone marrow.
67
What is acute promyelocytic leukemia sensitive to?
Acute promyelocytic leukemia is very sensitive to vitamin A.
68
What are the derivatives that contribute to excellent long-term survival in acute promyelocytic leukemia?
The derivatives are all-trans retinoic acid and arsenic.
69
From where can squamous-cell carcinomas originate?
Squamous-cell carcinomas can originate from the skin, the mucosa of the upper aerodigestive tract, and the cervix.
70
What are examples of locations where squamous-cell carcinomas can occur?
Examples include the head, neck, and esophagus.
71
What is the most appropriate dose of short-acting opioids for breakthrough pain in patients with uncontrolled cancer pain?
The most appropriate dose is 10% to 15% of the total daily opioid dose as needed every 3 to 4 hours.
72
What is vitamin B12 a cofactor for?
Vitamin B12 is a cofactor for converting methylmalonic acid into succinyl-CoA, which then goes into the Krebs cycle.
73
How is a poorly differentiated neoplasm diagnosed?
A poorly differentiated neoplasm is diagnosed on fine-needle aspiration biopsy.
74
What is required for a core biopsy?
A core biopsy requires immunohistochemical analysis.
75
What do guidelines recommend for patients with liver cirrhosis?
Patients with liver cirrhosis should undergo surveillance ultrasound every 6 months, with or without alpha-fetoprotein testing.
76
What is the reticulocyte count when anemia is caused by marrow infiltration by CLL cells?
The reticulocyte count is low.
77
What should a metaphyseal fracture in a child or young adult prompt evaluation for?
It should prompt evaluation for an underlying cause such as a primary bone tumor.
78
What is the recommended surveillance testing for patients with smoldering multiple myeloma?
Patients should undergo surveillance testing 2 to 3 months after initial diagnosis and if stable, monitored every 4 to 6 months for one year.
79
What are signs of superior vena cava syndrome?
Facial swelling, cough, and mediastinal widening on chest radiograph are signs of superior vena cava syndrome.
80
What is the final step in a root cause analysis?
The final step is to develop an action plan for system-wide change.
81
What can desmopressin reverse?
Desmopressin can reverse platelet dysfunction rapidly but transiently.
82
What is Gaucher disease?
Gaucher disease is the most common lysosomal storage disease caused by a deficiency of the glucocerebrosidase enzyme.
83
What are the symptoms associated with TTP?
TTP is associated with thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, acute kidney injury, and fever.
84
What causes Hemophilia B?
Hemophilia B is caused by a factor IX deficiency.
85
What condition is associated with tumor lysis syndrome?
Deficiency in uric acid levels >8 mg/dL despite aggressive volume expansion are candidates for treatment with rasburicase.
86
What are the symptoms associated with TTP?
TTP is associated with a pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, acute kidney injury, and fever.
87
What is the best laboratory test to diagnose adrenocorticotropic hormone-associated paraneoplastic syndrome?
An overnight dexamethasone suppression test or a 24-hour urinary free cortisol test.
88
What type of bladder cancer is associated with exposure to aromatic amine dyes?
Urothelial carcinoma.
89
What is the treatment of choice for patients with multiple brain metastases?
Whole-brain radiation plus glucocorticoid therapy.
90
What laboratory findings suggest autoimmune hemolytic anemia?
Evidence of hemolysis and peripheral spherocytosis.
91
What is the most common extracolonic type of cancer seen in women with Lynch syndrome?
Endometrial cancer.
92
What are other potential sites of malignancy associated with Lynch syndrome?
Stomach, small bowel, pancreas, hepatobiliary tract, ovaries, ureter, and renal pelvis.
93
Why must the mediastinum be assessed in a patient with NSCLC and lymphadenopathy?
It must be found free of tumor to proceed with curative resection.
94
What cancer is most typically associated with parathyroid hormone-related peptide-induced hypercalcemia?
Squamous-cell carcinoma of the lung.
95
What acute leukemia is very sensitive to all-trans retinoic acid?
Acute promyelocytic leukemia.
96
What happens to platelets after binding to vWF?
Platelets change their shape and release mediators that lead to activation of more platelets.
97
What is a common finding in a patient with hypercalcemia?
Proteinuria.
98
What can lead to the activation of more platelets?
Hypercalcemia, proteinuria, anemia, and kidney disease can lead to the activation of more platelets.
99
What should be evaluated in a patient with hypercalcemia, proteinuria, anemia, and kidney disease?
Evaluate for the diagnosis of multiple myeloma.
100
What long-term medications are associated with low levels of vitamin B12?
Long-term use of metformin or a proton pump inhibitor has been associated with development of low levels of vitamin B12.
101
When can prophylactic transfusion be considered?
Prophylactic transfusion can be considered in patients whose platelet count drops below 10,000 per mm3 since the risk of spontaneous bleeding is high below this level.
102
Does a warfarin overdose typically lead to persistent coagulopathy?
No, a warfarin overdose does not typically lead to persistent coagulopathy.
103
What should reverse warfarin therapy quickly?
Vitamin K should reverse warfarin therapy pretty quickly.
104
What is indicative of a diagnosis of a superior pulmonary sulcus (Pancoast) tumor?
The gradual development of arm weakness and Horner syndrome in an older patient with a history of cigarette smoking is most indicative of a diagnosis of a superior pulmonary sulcus (Pancoast) tumor.
105
What happens in a mixing study if the prolonged aPTT is caused by a lack of clotting factors?
The factors contained in the normal plasma will normalize the aPTT.
106
What may ITP patients who relapse or are unresponsive to dexamethasone respond to?
ITP patients who relapse or are unresponsive to dexamethasone may respond to intravenous immunoglobulin.
107
What can stimulate platelet production in ITP patients?
Eltrombopag or romiplostim can stimulate platelet production in ITP patients.
108
What is reserved for patients refractory to medical management?
Splenectomy is reserved for patients refractory to medical management.
109
What should patients with essential thrombocythemia and risk factors for complications be treated with?
Patients with essential thrombocythemia who have risk factors for complications should be treated with cytoreductive therapy with hydroxyurea.
110
How is immune thrombocytopenia (ITP) in people with HIV initially treated?
ITP in people with HIV is initially treated with antiretroviral therapy.
111
What treatments are added if ITP persists after virologic suppression?
If ITP persists after virologic suppression, other treatments such as glucocorticoids are added.
112
What is Von Hippel–Lindau disease?
Von Hippel–Lindau disease is a rare autosomal dominant familial cancer syndrome consisting chiefly of retinal angioma and hemangioblastoma of the central nervous system.
113
What is pheochromocytoma?
A tumor of the central nervous system.
114
What type of carcinoma is associated with clear-cell?
Clear-cell renal-cell carcinoma.
115
What occurs after platelet aggregation?
Formation of a white thrombus composed of platelets and fibrinogen.
116
What is TTP associated with?
A pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, acute kidney injury, and fever.
117
What is the most appropriate management for a patient taking apixaban with life-threatening bleeding?
Reversal with andexanet alfa or prothrombin complex concentrate.
118
In what clinical settings is reversal of anticoagulant recommended?
1. Bleeding at a critical site where there is a high risk that the function of a vital organ will be compromised with resulting disability or death.
2. Bleeding that is life-threatening.
3. Major bleeding that fails to respond to initial control measures.
119
What are critical bleeding sites?
Intracranial, thoracic, airway, pericardial, intra-abdominal (nongastrointestinal), retroperitoneal, intra-articular, and intramuscular bleeding.
120
What is the 4Ts score for HIT?
It takes into account the degree of thrombocytopenia, the timing of the decline in platelet count, the development of thrombosis or other sequelae, and the presence of other causes for thrombocytopenia.
121
What characterizes the 5q minus subtype of myelodysplastic syndrome?
Chronic transfusion-dependent anemia, which can lead to iron overload.
122
What is Epstein-Barr virus infection associated with?
Burkitt lymphoma, Hodgkin lymphoma, and nasopharyngeal carcinoma.
123
What are the clinical manifestations of PCT limited to?
The skin and liver.
124
What is Felty syndrome?
A condition that develops in about 1% of patients with rheumatoid arthritis, characterized by rheumatoid arthritis, splenomegaly, and neutropenia.
125
What lab findings are associated with anemia of chronic disease?
High ferritin level, low-normal iron level, and low total iron-binding capacity.
126
What is the likely diagnosis for anemia of chronic disease?
Anemia of chronic disease.
127
What is the goal of treatment for early-stage breast cancer?
To reduce local recurrence and breast cancer mortality among young women.
128
What is the treatment plan for young women with early-stage breast cancer treated with breast-conserving surgery?
The treatment plan should generally include irradiation of the breast.
129
What is the blast count for patients with accelerated-phase CML?
Patients have a blast count of 10% to 20%.
130
What is the blast count for patients with CML in blast crisis?
Patients have >20% blasts in the peripheral blood or bone marrow.
131
What is the first-line treatment for the 5q minus subtype of myelodysplastic syndrome?
The first-line treatment is lenalidomide.
132
What must be ruled out in essential thrombocythemia with a platelet count ≥1,000,000 per mm3?
Acquired von Willebrand syndrome must first be ruled out by measurement of ristocetin cofactor activity.
133
How does the left gonadal vein drain?
The left gonadal vein drains at a right angle into the left renal vein.
134
How does the right gonadal vein insert?
The right gonadal vein inserts directly into the inferior vena cava at a more acute angle, which makes drainage easier.
135
What is the diagnosis for leukocytosis above 100,000 cells per mm3 with absolute basophilia, thrombocytosis, and splenomegaly?
The most likely diagnosis is chronic myeloid leukemia.
136
What test is used to evaluate for von Willebrand disease brought on by aortic stenosis?
Von Willebrand multimer analysis.
137
What is the treatment for radiation pneumonitis?
The treatment is glucocorticoid therapy, usually consisting of a 2-week course of prednisone followed by a gradual taper.
138
What is the treatment for lobular carcinoma in-situ?
The treatment is surgical excision followed by tamoxifen administration.
139
What is the risk category for patients with acute lymphocytic leukemia and a leukocyte count >50,000 cells per mm3?
They are in the highest risk category for TLS.
140
What is squamous-cell carcinoma of the oropharynx associated with in a non-smoker?
It is most often associated with human papillomavirus infection, particularly HPV 16.
141
What is the most appropriate management approach for chronic immune thrombocytopenia in a patient with persistent asymptomatic moderate thrombocytopenia after splenectomy?
The approach is observation with no additional treatment.
142
How is asymptomatic localized Kaposi sarcoma managed?
It is managed by close observation after initiation of antiretroviral therapy.
143
What are patients who have undergone gastric bypass surgery at risk for?
They are at risk for iron deficiency.
144
What deficiencies are patients at risk for after gastric bypass surgery?
Patients who have undergone gastric bypass surgery are at risk for iron deficiency and vitamin B12 deficiency.
145
What develops as food bypasses iron absorption sites in the duodenum?
Iron deficiency anemia develops as food bypasses iron absorption sites in the duodenum.
146
What is the most appropriate approach to managing intractable back pain in a patient with metastatic cancer?
The most appropriate approach is to initiate bolus doses of intravenous opioids.
147
What symptoms are commonly associated with taxanes such as paclitaxel?
Taxanes such as paclitaxel are commonly associated with sensory neuropathy.
148
What characterizes TTP?
TTP is characterized by deficiency of ADAMTS13, a plasma protease that normally cleaves ultra-large von Willebrand factor multimers.
149
What is the most appropriate dose of short-acting opioids for breakthrough pain in patients with uncontrolled cancer pain?
The most appropriate dose is 10% to 15% of the total daily opioid dose, as needed every 3 to 4 hours.
150
What are topoisomerase inhibitors and give an example?
Topoisomerase inhibitors are chemotherapy agents such as doxorubicin.
151
What condition can lead to leukemia?
Leukemia can occur even earlier after treatment.
152
What characterizes reversible coagulopathy?
It is characterized by a high international normalized ratio and deficiencies of factors II, VII, IX, and X.
153
What should raise suspicion for superwarfarin intoxication?
Deficiencies of factors II, VII, IX, and X should raise suspicion for superwarfarin intoxication.
154
What are Auer rods associated with?
Auer rods are associated with disseminated intravascular coagulation and the chromosomal translocation t(15;17), which is acute promyelocytic leukemia.
155
What is the most likely diagnosis in a young man with severe anemia?
The most likely diagnosis is acquired pure red-cell aplasia.
156
What findings are associated with acquired pure red-cell aplasia?
Normal platelets and white blood cells, a low reticulocyte count, and a lack of erythroid precursors on bone-marrow examination.
157
What is the treatment for acquired pure red-cell aplasia?
Treatment generally involves immunosuppression.
158
What does PNH lead to?
PNH leads to the partial or complete absence of glycosylphosphatidylinositol-linked proteins, e.g., CD55 and CD59.
159
What is Von Hippel–Lindau disease?
Von Hippel–Lindau disease is a rare autosomal dominant familial cancer syndrome consisting chiefly of retinal angioma, hemangioblastoma of the central nervous system, pheochromocytoma, and clear-cell renal-cell carcinoma.
160
What does the diagnostic workup for squamous-cell carcinoma of the head and neck include?
It includes direct laryngoscopy, examination under anesthesia, and concurrent bilateral tonsillectomy.
161
What can lead to end-organ dysfunction?
Iron overload from transfusion-related etiologies can lead to end-organ dysfunction, especially in the liver, myocardium, and endocrine organs.
162
What are PNH cells susceptible to?
PNH cells are susceptible to complement-mediated lysis that occurs in the intravascular space and causes hemoglobinuria and hemosiderinuria.
163
What is G6PD deficiency?
G6PD deficiency is an X-linked recessive disease.
164
What is the best initial treatment for warm autoimmune hemolytic anemia?
The best initial treatment is a glucocorticoid.
165
What is W251bGwsIlpjMmUxZDJmODJlZWFmNTE0NjdjMmFkMjFiMjJhMGZjMCJd?
It is a glucocorticoid.
166
What should be considered with glucocorticoid therapy?
Concurrent or early initiation of rituximab.
167
What is a transfusion reaction?
It is defined as fever >38°C or an increase of at least 1°C from baseline that occurs during or up to 4 hours after a blood transfusion.
168
What is the treatment of choice for patients with multiple brain metastases?
Whole-brain radiation plus glucocorticoid therapy.
169
What is the follow-up for low-risk MGUS laboratory evaluation?
Labs can be repeated in approximately 3 months and then at 6- to 12-months.
170
What are common culprits for drug-induced immune-mediated thrombocytopenia?
Trimethoprim–sulfamethoxazole, quinine, vancomycin, and penicillin.
171
What causes Type 2N von Willebrand disease?
Mutations in von Willebrand factor that affect the binding site for factor VIII.
172
What is the most likely diagnosis in a patient with microcytic anemia and normal red-cell distribution width?
Beta-thalassemia trait.
173
What type of cancer is most associated with acanthosis nigricans?
Gastric cancer.
174
How does radiation-associated angiosarcoma typically manifest?
It manifests several years after therapy with a cutaneous tumor that looks similar to a bruise or hematoma.
175
What is the prognosis for patients with radiation-associated angiosarcoma?
Prognosis is usually poor as local and distant recurrence is common.
176
What is the risk for developing Kaposi sarcoma in patients with human herpesvirus 8 seropositivity?
They have a high risk if they undergo transplantation and immunosuppression.
177
What is the risk for cervical cancer in people with HIV?
It is much higher than in the general population, even in patients taking antiretroviral therapy.
178
What is the most likely diagnosis in a young man with HIV?
A rapidly progressive condition.
179
What is the most likely diagnosis in a young man with HIV presenting with a rapidly enlarging neck mass?
Burkitt lymphoma
180
What symptoms are associated with Burkitt lymphoma?
Night sweats and weight loss
181
What results from hereditary spherocytosis?
Diminished compliance of the cell and degradation by the spleen resulting in splenomegaly, anemia, and jaundice.
182
What does the diagnostic workup for squamous-cell carcinoma of the head and neck include?
Direct laryngoscopy, examination under anesthesia, and concurrent bilateral tonsillectomy.
183
What is the most appropriate initial therapy for a patient with newly diagnosed thrombotic thrombocytopenic purpura?
Therapeutic plasma exchange
184
What can copper deficiency manifest as?
Pancytopenia with macrocytic anemia
185
What should be considered as a likely diagnosis in a patient with urticaria pigmentosa and anaphylaxis in response to an antibiotic?
Systemic mastocytosis
186
What is a typical manifestation of renal-cell carcinoma?
Hematuria, flank pain, or a palpable abdominal mass
187
What is chronic HIV infection a well-characterized cause of?
Immune thrombocytopenic purpura (ITP)
188
What does the 4Ts score for HIT take into account?
1. Degree of thrombocytopenia, 2. Timing of the decline in platelet count, 3. Development of thrombosis or other sequelae, 4. Presence of other causes for thrombocytopenia.
189
What occurs after platelet aggregation?
Formation of a white thrombus composed of platelets and fibrinogen.
190
What are often present in chronic myeloid leukemia (CML)?
Thrombocytosis and splenomegaly
191
What is the most common initial test for HIT?
An enzyme-linked immunosorbent immunoassay (ELISA) that identifies the presence of antiplatelet factor 4 antibodies in serum.
192
What are the effects of tamoxifen?
Antiestrogenic effects in the breast and estrogenic effects in the uterus and bone, which may cause hyperplasia and worsen cancer.
193
What is the appropriate treatment to relieve symptoms of diarrhea and flushing associated with carcinoid syndrome?
A somatostatin analogue such as octreotide while controlling tumor growth.
194
What is marked anemia?
A significant decrease in red blood cells or hemoglobin levels.
195
What medication is associated with tumor lysis syndrome?
Octreotide
196
What does the presence of spherocytes in a patient with SLE suggest?
Autoimmune hemolytic anemia
197
What uric acid level indicates the need for treatment with rasburicase?
Uric acid levels >8 mg/dL despite aggressive volume expansion
198
Is amyloidosis associated with massive hepatosplenomegaly?
Amyloidosis is not associated with massive hepatosplenomegaly
199
Where do most CNS lymphomas cluster on imaging?
Supratentorially with a predilection for the deep gray matter of the frontal and parietal lobes
200
What should a fracture in a long bone of a young adult prompt evaluation for?
Osteosarcoma
201
What is the most common primary bone tumor affecting children and young adults?
Ewing sarcoma
202
What is the recommended screening for people aged 50 to 80 with a history of smoking?
Annual screening with low-dose chest CT
203
What are major clinical manifestations of mixed cryoglobulinemic vasculitis?
Palpable purpura, kidney disease, arthralgia, myalgia, peripheral neuropathy
204
At what age can screening mammography be discontinued?
Approximately 75 years of age
205
What is typical of chronic lymphocytic leukemia?
Peripheral lymphocytosis with small lymphocytes
206
Does early treatment of asymptomatic patients with CLL improve outcomes?
No, it does not improve outcomes
207
What are patients with lymphoma at risk for?
Developing a rapidly enlarging mediastinal mass with airway obstruction
208
What activates factor VII in the extrinsic pathway of coagulation?
Tissue factor (factor III)
209
What is CLL characterized by?
CLL has an increase in mature appearing B lymphocytes.
210
What activates factor VII in the extrinsic pathway of coagulation?
Tissue factor (factor III) activates factor VII.
211
What are meningiomas?
Meningiomas are diffusely enhancing lesions that occur most commonly in the parasellar regions of the base of the skull or cerebral convexities.
212
Where do most CNS lymphomas cluster?
Most CNS lymphomas cluster around the ventricles with a predilection for the deep gray matter of the frontal and parietal lobes.
213
What is the most likely diagnosis in a patient with pancytopenia without myeloid immaturity?
The most likely diagnosis is Gaucher disease.
214
What is Gaucher disease?
Gaucher disease is the most common lysosomal storage disease, an autosomal recessive disorder caused by a deficiency of the glucocerebrosidase enzyme, leading to the accumulation of glycolipids.
215
Why is iron deficiency common in dialysis patients?
Iron deficiency is common in dialysis patients because of frequent blood testing and blood lost from the procedure.
216
Are carcinoid tumors typically associated with urticarial skin lesions?
Carcinoid tumors are not typically associated with urticarial skin lesions.
217
What are the functional assays that test the ability of a patient's HIT antibodies?
The heparin-induced platelet aggregation (HIPA) assay and the serotonin release assay (SRA) are functional assays that test the ability of a patient's HIT antibodies to activate test platelets.
218
What is the first-line therapy for hemolytic PNH?
The first-line therapy for hemolytic PNH is eculizumab, a monoclonal antibody.
219
What is a monoclonal antibody?
A monoclonal antibody is an antibody produced from a single clone of cells, designed to target a specific antigen.
220
What does a monoclonal antibody target?
It targets complement protein C5.
221
What is included in the appropriate evaluation of adenocarcinoma in an axillary lymph node?
Bilateral diagnostic mammography with or without ultrasound of the breast.
222
What does lupus anticoagulant lead to?
It leads to a prolonged prothrombin time or partial-thromboplastin time.
223
What is the most likely cause of a new-onset bleeding disorder in a previously healthy postpartum woman?
Acquired factor VIII inhibitor.
224
What is the role of Factor VIIa and tissue factor?
They form a complex that requires factor IV for calcium.
225
What is the best initial treatment for warm autoimmune hemolytic anemia?
A glucocorticoid with consideration for concurrent or early initiation of rituximab.
226
What causes cold agglutinin hemolytic anemia?
IgM antibodies bind to polysaccharides on erythrocytes.
227
What are common culprits for drug-induced immune-mediated thrombocytopenia?
Trimethoprim-sulfamethoxazole, quinine, vancomycin, and penicillin.
228
What can benefit a patient with kidney failure and hyperuremia?
Desmopressin administration can increase vWF-factor 8 multimers.
229
What is indicated by leukocytosis above 100,000 cells per mm3?
It may be associated with absolute basophilia and thrombocytosis.
230
What is the cell count indicating absolute basophilia?
100,000 cells per mm3
231
What are common findings in chronic myeloid leukemia?
Thrombocytosis and splenomegaly
232
What is the most likely diagnosis with thrombocytosis and splenomegaly?
Chronic myeloid leukemia
233
What infections can cause cold agglutinin hemolytic anemia?
Mycoplasma or Epstein–Barr virus
234
What is Von Hippel–Lindau disease?
A rare autosomal dominant familial cancer syndrome consisting chiefly of retinal angioma, hemangioblastoma, pheochromocytoma, and clear-cell renal-cell carcinoma.
235
What is the most common cause of death in patients with PNH?
Thrombosis
236
What condition can some patients with PNH experience?
Bone-marrow failure resulting in pancytopenia
237
What is PNH associated with?
Increased risk for myelodysplastic syndrome, aplastic anemia, and leukemia.
238
What percentage of GISTs have mutations in proto-oncogene KIT?
Approximately 80%
239
What is Burkitt lymphoma?
An aggressive non-Hodgkin lymphoma associated with c-myc translocation usually in association with Epstein–Barr virus infection.
240
What factors are thought to precipitate PCT?
Multiple risk factors contributing to iron accumulation and/or oxidative stress in hepatocytes.
241
How can renal-cell carcinoma typically manifest?
As hematuria, flank pain, or a palpable abdominal mass.
242
What is the most appropriate medication for preventing thromboembolism in a patient with nonvalvular atrial fibrillation?
Not specified in the provided text.
243
What are risk factors associated with chronic kidney disease?
Warfarin is a risk factor associated with chronic kidney disease.
244
What can radiotherapy for prostate cancer lead to?
Radiotherapy for prostate cancer can lead to delayed bowel injury known as chronic radiation proctitis.
245
When does vancomycin-induced thrombocytopenia often start?
Vancomycin-induced thrombocytopenia often starts after around 5 days of treatment with a mean nadir platelet count of 13.
246
What is the mean nadir platelet count in vancomycin-induced thrombocytopenia?
The mean nadir platelet count is 600 per mm3 occurring 8 days after initiation of treatment.
247
Can granulocyte colony-stimulating factor be given to patients with CLL?
Granulocyte colony-stimulating factor can be given to patients with CLL if they also have neutropenia.
248
What is the most appropriate first-line treatment for stroke prevention in a patient with sickle cell disease?
The most appropriate first-line treatment for stroke prevention in a patient with sickle cell disease and a history of transient ischemic attacks is not specified.
249
What is prophylactic red-cell exchange transfusions?
Prophylactic red-cell exchange transfusions are a medical procedure used to prevent complications in patients.
## Footnote
No back content.
250
What does a mixing study reveal if the prolonged aPTT is caused by a lack of clotting factors?
In a mixing study, if the prolonged aPTT is caused by a lack of clotting factors, the factors contained in the normal plasma will normalize the aPTT.
## Footnote
No back content.
251
What is celiac plexus neurolysis?
Celiac plexus neurolysis is a percutaneous or endoscopic ultrasound-guided method for achieving pain control.
## Footnote
No back content.
252
What is the purpose of celiac plexus neurolysis?
It is used for pain control in patients with pancreatic cancer or other cancers of the upper abdomen.
## Footnote
The organs of the upper abdomen typically send nociceptive pain signals through the celiac plexus to the spinal cord.
253
What substances are injected during celiac plexus neurolysis?
During celiac plexus neurolysis, alcohol or phenol is injected to destroy the nerve tissue.
## Footnote
This reduces the transmission of pain signals.
254
What percentage of patients with pain from pancreatic cancer experience significant improvement after celiac plexus neurolysis?
Approximately 70% to 75% of patients with pain from pancreatic cancer experience a significant improvement in pain after this procedure.
## Footnote
Although most patients continue to experience some pain.
255
What is thrombotic thrombocytopenic purpura classically associated with?
Thrombotic thrombocytopenic purpura is classically associated with microangiopathy, thrombocytopenia, renal insufficiency, mental status changes, and fever.
## Footnote
No back content.
256
What are the first-line treatments for symptomatic anemia in patients with low-risk myelodysplastic syndrome?
The most appropriate first-line treatments are erythropoietin and transfusion support.
## Footnote
No back content.
257
What is the classic appearance of meningiomas on imaging?
Meningiomas are adjacent to bone and anchored to the dura, rendering the classic appearance of the 'dural tail' on imaging.
## Footnote
No back content.
258
Are adults with mild sickle cell disease likely to have splenomegaly?
Adults with even mild sickle cell disease are unlikely to have splenomegaly because they experience splenic infarcts during crises.
## Footnote
The splenomegaly is an important hint to the presence of SC disease.
259
What are the symptoms of acute porphyria?
Symptoms include severe abdominal pain, sensory and peripheral neuropathies, neuropsychiatric abnormalities, and red-tinged urine.
## Footnote
Diagnostic Test: urine test for porphobilinogen.
260
What medications will precipitate acute porphyria?
Sulfonamides, barbiturates, and hydantoins (phenytoin) will precipitate acute porphyria.
## Footnote
No back content.
261
What is neurolysis?
Neurolysis is a percutaneous or endoscopic ultrasound-guided method for achieving pain control in patients with pancreatic cancer or other cancers of the upper abdomen.
262
How do the organs of the upper abdomen send pain signals?
The organs of the upper abdomen typically send nociceptive pain signals through the celiac plexus to the spinal cord.
263
What substances are injected during celiac plexus neurolysis?
During celiac plexus neurolysis, alcohol or phenol is injected, destroying the nerve tissue and thus reducing transmission of pain signals.
264
What percentage of patients with pain from pancreatic cancer experience significant improvement after celiac plexus neurolysis?
Approximately 70% to 75% of patients with pain from pancreatic cancer experience a significant improvement in pain after this procedure.
265
What is transfusion-associated lung injury?
Transfusion-associated lung injury usually manifests 1 to 6 hours after the transfusion is initiated.
266
In which individuals does chronic lymphocytic leukemia (CLL) occur more frequently?
Chronic lymphocytic leukemia (CLL) occurs more frequently in older individuals.
267
What indicates functional iron deficiency in patients with chronic kidney failure?
Functional iron deficiency is likely in patients with chronic kidney failure if they have a ferritin level of ≤500 ng/mL or a transferrin saturation of ≤30%.
268
How should patients with chronic lymphocytic leukemia and serious recurrent infections be treated?
Patients with chronic lymphocytic leukemia and serious recurrent infections should be treated with intravenous immunoglobulin.
269
What is hereditary hemochromatosis (HH) commonly caused by?
Hereditary hemochromatosis (HH) is most commonly caused by mutations in the HFE gene, such as C282Y or less commonly H63D.
270
What are typical manifestations of renal-cell carcinoma?
Renal-cell carcinoma can typically manifest as hematuria, flank pain, or a palpable abdominal mass.
271
When should first-degree relatives of patients who develop colorectal cancer start screening?
First-degree relatives of patients who develop colorectal cancer before age 60 should start screening at either age 40 or 10 years before the age at which the index case was diagnosed, whichever comes first.
272
What are major clinical manifestations of mixed cryoglobulinemic vasculitis?
Major clinical manifestations of mixed cryoglobulinemic vasculitis include palpable purpura.
273
What is palpable purpura?
Palpable purpura is a type of skin rash characterized by raised purple spots.
## Footnote
Example: It can be associated with various conditions, including vasculitis.
274
What conditions can kidney disease lead to?
Kidney disease can lead to arthralgia and myalgia, as well as peripheral neuropathy.
275
What is hypocomplementemia?
Hypocomplementemia is a condition where complement levels in the blood are low.
## Footnote
Example: It can be associated with certain autoimmune diseases.
276
What are common causes of paraneoplastic syndrome?
Bronchial carcinoid tumors are among the most common causes of paraneoplastic syndrome.
277
What is Cushing syndrome?
Cushing syndrome is a condition caused by excess cortisol in the body.
278
What is therapy-related acute myeloid leukemia?
Therapy-related acute myeloid leukemia classically develops 5 to 7 years after treatment with alkylating chemotherapy agents and radiotherapy.
279
What does a periosteal reaction indicate?
A periosteal reaction is indicative of Ewing sarcoma.
280
What is the management for life-threatening bleeding in patients taking apixaban?
The management includes reversal with andexanet alfa or prothrombin complex concentrate.
281
What is celiac plexus neurolysis?
Celiac plexus neurolysis is a method for achieving pain control in patients with pancreatic cancer or other cancers of the upper abdomen.
282
What is the purpose of injecting alcohol or phenol during celiac plexus neurolysis?
Injecting alcohol or phenol destroys nerve tissue, thus reducing the transmission of pain signals.
283
What percentage of patients with pain from pancreatic cancer experience improvement after celiac plexus neurolysis?
Approximately 70% to 75% of patients with pain from pancreatic cancer experience a significant improvement in pain after this procedure.
284
What is the best approach to breast cancer screening for young women with a high lifetime risk?
The best approach is annual MRI in addition to annual mammography.
285
Why should both MRI and mammography be performed annually?
Both screening tests should be performed annually as MRI has better sensitivity in young women.
286
What imaging method has better sensitivity in young women with increased mammographic density?
Mammography is superior to MRI in detecting microcalcifications associated with ductal carcinoma in situ.
287
What is associated with reductions in bone mineral density during androgen-deprivation therapy?
Reductions can occur as early as the first 6 to 12 months of treatment.
288
What is the most prominent clinical finding of thrombocytopenia?
The presence of petechiae.
289
In what percentage of patients with cryoglobulin vasculitis is the disorder associated with chronic hepatitis C virus infection?
About 70%.
290
What are other causes of cryoglobulin vasculitis?
Other viruses, including hepatitis B virus, Epstein–Barr virus, or HIV.
291
What is the most likely diagnosis in a patient with pancytopenia and Auer rods?
Acute promyelocytic leukemia.
292
What activates factor VII in the extrinsic pathway of coagulation?
Tissue factor (factor III).
293
What anticoagulants have the most evidence to support their efficacy in preventing recurrent venous thromboembolism in cancer patients?
Low-molecular-weight heparin and direct oral factor Xa inhibitors.
294
What is characteristic of hairy-cell leukemia?
Pancytopenia in association with splenomegaly, B symptoms, monocytopenia, and atypical lymphocytes with projections.
295
What should be considered as a likely diagnosis in a patient with both urticaria pigmentosa and anaphylaxis in response to an antibiotic?
Systemic mastocytosis.
296
Why is thrombocytosis often seen in beta thalassemia patients?
Due to increased drive on the marrow from erythropoietin.
297
What is the most appropriate treatment for patients with chemotherapy-induced nausea and vomiting who do not respond to preventive treatment with prochlorperazine?
Intravenous palonosetron and dexamethasone.
298
What is the appropriate treatment for 3NSJd?
Intravenous palonosetron and dexamethasone.
299
What are the clinical manifestations of PCT limited to?
The skin and liver.
300
What percentage of GISTs have mutations in the proto-oncogene KIT?
Approximately 80%.
301
What type of skeletal metastases are predominant in certain cancers?
Osteoblastic or sclerotic skeletal metastases.
302
What should raise suspicion for metastatic prostate cancer?
Adenocarcinoma of an unknown primary site in a man.
303
What is the best initial management for patients with metastatic cancer admitted with intractable pain?
A patient-controlled analgesia pump with an intravenous opioid.
304
What features are associated with increased risk for progression of MGUS?
1. A serum monoclonal protein level >1.5 g/dL
2. Non–immunoglobulin G monoclonal protein
3. A markedly abnormal free-light-chain-kappa-to-lambda ratio.
305
What is the best strategy for managing cancer-related pain that is intractable despite intermittent opioids?
Intravenous opioids administered as patient-controlled analgesia.
306
What is the continuous infusion rate for patient-controlled analgesia?
50% to 100% of the patient’s 24-hour opioid intake.
307
What is Porphyria cutanea tarda (PCT) caused by?
An acquired deficiency of hepatic uroporphyrinogen decarboxylase.
308
What are myelocytes precursors to?
Basophils, eosinophils, and neutrophils.
309
What does PNH lead to?
The partial or complete absence of glycosylphosphatidylinositol-linked proteins, e.g., CD55 and CD59.
310
What is the most effective management option for symptomatic progressive prostate cancer in older men with a life expectancy of <10 years?
Radiation therapy with consideration for androgen-deprivation therapy.
311
How can the risk of progression from asymptomatic to symptomatic MM be estimated?
Using the Mayo Clinic 2018 risk stratification system.
312
What are the three risk factors considered in the Mayo Clinic 2018 risk stratification system?
1. M protein >2 g/dL
2. Bone marrow.
313
What is the M protein level indicative of?
M protein >2 g/dL
314
What percentage of clonal plasma cells in the bone marrow indicates a condition?
Bone marrow clonal plasma cells >20%
315
What is the involved–uninvolved free-light-chain ratio threshold?
Involved–uninvolved free-light-chain ratio >20
316
What risk factors categorize patients with smoldering MM?
Patients are considered to be at low risk for smoldering MM if they have no risk factors, intermediate risk if they have one risk factor, or high risk if they have 2 or 3 risk factors.
317
What is the initial treatment for immune thrombocytopenia (ITP) in people with HIV?
ITP is initially treated with antiretroviral therapy.
318
What treatments are added if ITP persists after virologic suppression?
Other treatments such as glucocorticoids are added.
319
What is the first-line medication for reducing acute pain episodes in sickle cell disease?
The first-line medication is hydroxyurea.
320
Why are varicoceles common on the left side?
Varicoceles are fairly common on the left side because of the higher pressure within the left gonadal vein compared with the right gonadal vein.
321
What is the best screening for a patient with a sibling who has a history of acute myeloid leukemia?
The best screening is routine medical care.
322
How is acute chest syndrome defined?
Acute chest syndrome is defined as chest pain associated with a new opacity on chest radiography and associated fever or respiratory distress.
323
What is the risk of progression from asymptomatic to symptomatic MM estimated by?
The Mayo Clinic 2018 risk stratification system.
324
What are the three risk factors considered in the Mayo Clinic risk stratification for MM?
1. M protein >2 g/dL
2. Bone marrow clonal plasma cells >20%
3. Involved–uninvolved free-light-chain ratio >20
325
What risk level are patients considered if they have no risk factors for smoldering MM?
Low risk.
326
What risk level are patients considered if they have one risk factor for smoldering MM?
Intermediate risk.
327
What risk level are patients considered if they have 2 or 3 risk factors for smoldering MM?
High risk.
328
What is likely the diagnosis for severe thrombocytopenia in a patient with no signs of bruising or bleeding?
Pseudothrombocytopenia.
329
What causes pseudothrombocytopenia?
Naturally occurring antibodies directed against an epitope on glycoprotein IIb/IIIa.
330
What anticoagulant should not be discontinued in patients at risk for recurrent thrombosis?
Warfarin.
331
What is the best strategy to manage warfarin in a patient needing a minor procedure?
Continue the anticoagulant therapy.
332
What are examples of minor procedures where anticoagulant therapy can be continued?
Tooth extraction, excision of basal- or squamous-cell carcinoma, and cataract surgery.
333
What is Von Hippel–Lindau disease?
A rare autosomal dominant familial cancer syndrome consisting chiefly of retinal angioma, hemangioblastoma of the central nervous system, pheochromocytoma, and clear-cell renal-cell carcinoma.
334
What do current guidelines recommend for screening for lung cancer?
Annual screening with low-dose chest CT for people age 50 to 80 years with a history of at least 20 pack-years of cigarette smoking.
335
What is the most appropriate treatment for a single small (<3.5 cm) brain metastasis in a patient with unresectable non–small-cell lung cancer?
Stereotactic radiosurgery.
336
What initiates the extrinsic pathway of coagulation?
Factor VIIa.
337
What initiates the extrinsic pathway of coagulation?
Factor VIIa and tissue factor form a complex.
338
What effect does Bupropion have on seizures?
Bupropion can lower seizure threshold.
339
What process does methionine undergo?
Methionine converts into homocysteine, then into cystathionine. Vitamin B12 can be used to recycle it back into methionine.
340
What inheritance pattern do autosomal dominant diseases exhibit?
They have incomplete penetrance and may occasionally appear to skip a generation.
341
What are the diagnostic criteria for MGUS?
Monoclonal protein detected in serum at a level <3.0 g/dL; <10% plasma cells in bone marrow; absence of hypercalcemia, renal failure, anemia, and lytic bone lesions.
342
What is the best strategy for managing cancer-related pain?
Intravenous opioids administered as patient-controlled analgesia with a continuous infusion rate equal to 50% to 100% of the patient's 24-hour opioid intake.
343
How does the left gonadal vein drain?
The left gonadal vein drains at a right angle into the left renal vein.
344
How should a warfarin-treated patient with an elevated INR >10 be managed?
Administer low-dose vitamin K by mouth and withhold warfarin.
345
Where do gastrointestinal stromal tumors (GIST) commonly occur?
GIST can occur throughout the gastrointestinal tract, most commonly in the stomach.
346
What is a potential complication of oral agents that inhibit tyrosine kinase activity?
Interstitial lung disease is an uncommon but potentially fatal complication.
347
What causes Type 2N von Willebrand disease?
It is caused by mutations in von Willebrand factor that affect the binding site for factor VIII, leading to impaired binding and rapid clearance of the factor.
348
What can lead to impaired binding and rapid clearance of a factor in a patient with hypercalcemia?
Proteinuria, anemia, and kidney disease
## Footnote
Evaluate for the diagnosis of multiple myeloma.
349
What is Beck triad associated with?
Pericardial effusion
## Footnote
It includes low systolic blood pressure, jugular venous distention, and muffled heart sounds.
350
What is the most likely cause of normocytic normochromic anemia in a 35-year-old woman with bradycardia and menometrorrhagia?
Hypothyroidism.
351
How can patients with superwarfarin intoxication be managed?
With fresh frozen plasma and vitamin K
## Footnote
Followed by several months of tapering vitamin K therapy until the agent is eliminated from the body.
352
What is beta-thalassemia?
A congenital hemolytic anemia caused by reduced or absent synthesis of the beta chains of hemoglobin.
353
What are the phenotypes of beta-thalassemia?
They vary considerably, including asymptomatic carrier state (beta-thalassemia minor), moderate anemia, and severe transfusion-dependent anemia (beta-thalassemia major).
354
What must be ruled out in essential thrombocythemia if the platelet count is ≥1,000,000 per mm3?
Acquired von Willebrand syndrome
## Footnote
Must be ruled out by measurement of ristocetin cofactor activity.
355
What are the major criteria for Polycythemia Vera (PV)?
1. Elevated hemoglobin level (>16.0 g/dL in women; >16.5 g/dL in men) or elevated hematocrit (>48% in women; >49% in men).
2. Bone-marrow biopsy consistent with PV.
3. Presence of the JAK2 V617F or another similar mutation.
356
What is a minor criterion for Polycythemia Vera (PV)?
Serum erythropoietin level below the normal reference range.
357
What is the significance of levels below the normal reference range?
It indicates a condition that may require further investigation, especially in most CNS cases.
358
Where do lymphomas typically cluster in the brain?
Lymphomas are supratentorial and cluster around the ventricles with a predilection for the deep gray matter of the frontal and parietal lobes.
359
What does beta-thalassemia lead to?
Beta-thalassemia leads to abnormal erythrocytes that express procoagulant phospholipids, activated clotting factors, and activated platelets, all of which predispose patients to thrombosis.
360
What tumor marker is most likely elevated in a yolk sac tumor?
The tumor marker most likely to be elevated in a yolk sac tumor is alpha-fetoprotein.
361
Why isn't Von Willebrand's disease the likely diagnosis for a young woman with excessive bleeding gums post-partum?
Von Willebrand's disease is inherited; the absence of family and past history of bleeding makes it unlikely.
362
What is the association of EBV with cancers?
EBV is associated with nasopharyngeal cancers, Burkitt lymphoma, and Hodgkin lymphoma.
363
What is the most likely diagnosis in a patient with microcytic anemia and normal red-cell distribution width?
The most likely diagnosis is beta-thalassemia trait.
364
What is the blast count in patients with accelerated-phase CML?
Patients with accelerated-phase CML have a blast count of 10% to 20%.
365
What characterizes patients with CML in blast crisis?
Patients with CML in blast crisis have more than 20% blasts in the peripheral blood or bone marrow.
366
What deficiencies are patients at risk for after gastric bypass surgery?
Patients who have undergone gastric bypass surgery are at risk for iron deficiency and vitamin B12 deficiency.
367
What causes vitamin B12 deficiency after gastric bypass?
Vitamin B12 deficiency develops because of inadequate binding by intrinsic factor after gastric bypass.
368
What causes macrocytic anemia?
G6PD-deficient erythrocytes in combination with high levels of oxidants cause a cross-linking of sulfhydryl groups on globin chains; this causes their denaturation and the formation of Heinz bodies.
369
What is tumor lysis syndrome (TLS)?
TLS is caused by the release of the contents of malignant cells into the bloodstream resulting in electrolyte abnormalities.
370
What are some electrolyte abnormalities associated with TLS?
These may include hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia.
371
What is the triad of the most prominent clinical features of paroxysmal nocturnal hemoglobinuria?
The triad includes hemolytic anemia, thrombosis, and pancytopenia.
372
What is the most appropriate initial management approach for an asymptomatic patient with early-stage chronic lymphocytic leukemia?
Observation.
373
What is the most appropriate test to confirm a suspected diagnosis of hepatocellular cancer in a patient with cirrhosis?
Dynamic contrast-enhanced MRI or multiphasic CT of the liver.
374
What is the treatment of TLS?
Treatment includes rasburicase, aggressive volume expansion with normal saline, and if needed, a loop diuretic to achieve a target urine output of around 150–200 mL/hour for most adults.
375
What are the characteristics of very early stages of cervical cancer?
They are often asymptomatic and squamous-cell carcinomas comprise about 80% of all cases.
376
When may platelet-specific antibody testing be considered?
It may be considered in patients who fail to respond to HLA-compatible or cross-matched platelets and in patients at risk for post-transfusion complications.
377
What are platelets associated with in patients at risk for post-transfusion purpura?
Platelets are associated with the risk of post-transfusion purpura.
378
What are the prominent clinical features of paroxysmal nocturnal hemoglobinuria?
The triad of the most prominent clinical features includes hemolytic anemia, thrombosis, and pancytopenia.
379
What is thrombotic thrombocytopenic purpura classically associated with?
Thrombotic thrombocytopenic purpura is classically associated with microangiopathy.
380
What are the symptoms of superior vena cava syndrome?
Symptoms include a mediastinal mass, mental status changes, and fever.
381
How is hairy cell leukemia diagnosed?
Diagnosis requires soluble CD25 in serum and surface expression of CD103.
382
What is the first step in managing a patient with superior vena cava syndrome?
The first step is a biopsy.
383
What mediates platelet aggregation?
Platelet aggregation is mediated by GpIIb/IIIa-receptor and fibrinogen.
384
What is elevated in folate deficiency?
Homocysteine level is elevated, but not the methylmalonic acid level.
385
What does the treatment of TLS include?
The treatment includes rasburicase and aggressive volume expansion with normal saline.
386
What is the target urine output for most adults in TLS management?
The target urine output is around 150–200 mL/hour.
387
What can help diagnose HIT?
Testing for platelet factor 4 antibodies can be helpful in diagnosing HIT.
388
At what age can screening mammography be discontinued?
Screening mammography can be discontinued at approximately 75 years of age.
389
Where are most CNS lymphomas located?
Most CNS lymphomas are supratentorial, clustering around the ventricles with a predilection for the deep gray matter.
390
What causes pseudothrombocytopenia?
Pseudothrombocytopenia is due to EDTA altered platelet autoantibodies resulting in clumping of platelets.
391
What suggests autoimmune hemolytic anemia?
Evidence of hemolysis and peripheral spherocytosis suggests autoimmune hemolytic anemia.
392
What is a typical presentation of cervical cancer?
Irregular bleeding exacerbated by sexual intercourse is a typical presentation.
393
What are the major clinical manifestations of mixed cryoglobulinemic vasculitis?
Major clinical manifestations include palpable purpura, kidney disease, arthralgia, myalgia, and peripheral neuropathy.
394
What is arthralgia?
Arthralgia refers to joint pain.
395
What is myalgia?
Myalgia refers to muscle pain.
396
What is peripheral neuropathy?
Peripheral neuropathy is a condition resulting from damage to the peripheral nerves.
397
What is hypocomplementemia?
Hypocomplementemia is characterized by low levels of complement proteins in the blood.
398
What is chronic lymphocytic leukemia (CLL)?
Chronic lymphocytic leukemia is characterized by an increase of mature-appearing B lymphocytes.
399
What is therapy-related acute myeloid leukemia?
Therapy-related acute myeloid leukemia classically develops 5 to 7 years after treatment with alkylating chemotherapy agents and radiotherapy.
400
What are elevated levels of methylmalonic acid and homocysteine indicative of?
Elevated levels of methylmalonic acid and homocysteine are indicative of vitamin B12 deficiency.
401
What type of melanoma accounts for fewer than 5% of melanomas?
Acral lentiginous melanoma accounts for fewer than 5% of melanomas.
402
What is the most common type of melanoma in dark-skinned people?
Acral lentiginous melanoma is the most common type of melanoma in dark-skinned people.
403
Where is acral lentiginous melanoma typically found?
Acral lentiginous melanoma is found on the palms, soles, and occasionally in mucous membranes.
404
What is the initial therapy for heavy menstrual bleeding with hemodynamically significant hemorrhage?
The initial therapy is red-blood-cell transfusion.
405
What does the TF-FVIIa complex activate?
The TF-FVIIa complex activates factor X and factor IX.
406
What happens in the presence of a clotting inhibitor such as lupus anticoagulant?
The clotting time (aPTT) remains increased.
407
What triggers hemolytic anemia in patients with G6PD deficiency?
Hemolytic anemia is often triggered by exposure to medications.
408
What is polycythemia vera?
Polycythemia vera is a myeloproliferative neoplasm characterized by increased red-cell mass and elevated hemoglobin.
409
What symptoms are associated with polycythemia vera?
Symptoms include erythromelalgia (red, hot, painful extremities) and pruritus.
410
What is the role of von Willebrand factor (vWF) in platelet hemostasis?
After binding to vWF, platelets change their shape and release mediators that activate more platelets.
411
What should postmenopausal women with vaginal bleeding and a history of tamoxifen use undergo?
They should undergo evaluation with in-office endometrial biopsy.
412
What is the most appropriate management for severe abdominal pain in a patient with pancreatic cancer developing adverse effects from high doses of opioids?
The most appropriate management is neurolysis, such as a celiac plexus block.
413
What is neurolysis?
Neurolysis is a procedure such as a celiac plexus block.
414
What is a 24-hour urine sample used for?
A 24-hour urine sample for 5-hydroxyindoleacetic acid would be used to diagnose and monitor a carcinoid tumor.
415
How often should patients with smoldering multiple myeloma be monitored?
Patients whose smoldering MM is being managed with observation alone should undergo surveillance testing 2 to 3 months after initial diagnosis. If stable, the patient should be monitored every 4 to 6 months for one year.
416
What is the follow-up for stable patients with smoldering multiple myeloma?
Stable patients who remain asymptomatic without evidence of end-organ damage can then be followed every 6 to 12 months.
417
What is the most likely diagnosis in a person with HIV and thrombocytopenia?
Immune thrombocytopenia is the most likely diagnosis in a person with HIV whose peripheral-blood smear shows thrombocytopenia with a few large platelets.
418
When does heparin-induced thrombocytopenia usually occur?
HIT usually occurs 5 to 14 days after exposure (earlier in those with prior heparin exposure) and does not generally reduce platelet counts to below 10,000 per mm3.
419
What is the most appropriate next step for managing cancer pain that persists despite high doses of one opioid?
The most appropriate next step is to rotate to another opioid.
420
When does vancomycin-induced thrombocytopenia typically start?
Vancomycin-induced thrombocytopenia often starts after around 5 days of treatment, with a mean nadir platelet count of 13,600 per mm3 occurring 8 days after initiation of treatment.
421
What is the most likely diagnosis in an asymptomatic person with a mean corpuscular volume in the 60s?
The most likely diagnosis is beta-thalassemia trait or alpha-thalassemia trait.
422
What are the symptoms associated with TTP?
TTP is associated with a pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, acute kidney injury, and fever.
423
What is the recommended follow-up for a smoker with a single solid pulmonary nodule?
A follow-up low-dose CT scan of the chest in 6 months is recommended.
424
What levels are elevated in patients with vitamin B12 deficiency?
Methylmalonic acid and homocysteine levels are elevated in patients with vitamin B12 deficiency.
425
What suggests a diagnosis of hemoglobin SC disease?
The combination of mild anemia and infrequent pain crises in a patient with splenomegaly suggests a diagnosis of hemoglobin SC disease.
426
What is the best strategy to manage acute severe pain?
The best strategy to manage acute severe pain is not specified in the provided text.
427
What does a diagnosis of hemoglobin SC disease suggest?
It suggests a specific type of hemoglobinopathy.
428
What is the best strategy to manage acute severe pain at the end of life?
Bolus doses of intravenous opioids.
429
What is required for the diagnosis of systemic mastocytosis?
A bone biopsy.
430
What condition is indicated by hereditary hemochromatosis (HH)?
It is most commonly caused by mutations in the HFE gene.
431
What are the common mutations associated with hereditary hemochromatosis?
C282Y or less commonly H63D.
432
In which demographic does hereditary hemochromatosis typically manifest?
In middle-aged white men, often of Irish heritage.
433
What are the clinical features of hereditary hemochromatosis?
Gradually progressive adenopathy and splenomegaly.
434
What systemic symptoms are concerning for lymphoma?
Fatigue, fever, night sweats, and weight loss.
435
What should clinicians always exclude in febrile transfusion reactions?
Hemolysis.
436
What test should be performed to check for hemolysis in transfusion reactions?
A direct antiglobulin test.
437
What medication frequently causes neutropenia?
Ganciclovir.
438
What are the major criteria for Polycythemia Vera (PV)?
1. Elevated hemoglobin level (>16.0 g/dL in women; >16.5 g/dL in men). 2. Elevated hematocrit (>48% in women; >49% in men). 3. Bone-marrow biopsy consistent with PV.
439
What is a minor criterion for Polycythemia Vera?
Serum erythropoietin level below the normal reference range.
440
How can TACO be distinguished from TRALI?
By the presence of hypertension and distended neck veins suggesting increased central venous pressure.
441
What is the increased risk of cancer in patients with Common Variable Immunodeficiency (CVID)?
Particularly non-Hodgkin lymphoma.
442
What is the most appropriate treatment for transfusion-induced iron overload with evidence of end-organ dysfunction?
Iron chelation therapy with a treatment such as deferasirox.
443
What are the most common acquired risk factors for Budd-Chiari syndrome?
Myeloproliferative neoplasms.
444
What is the typical treatment for radiation-associated angiosarcoma?
Mastectomy is preferred.
445
What is the typical treatment for Radiation-associated angiosarcoma?
Mastectomy is typically preferred.
446
What is the most likely diagnosis in a patient with microcytic anemia and normal red-cell distribution width?
Beta-thalassemia trait.
447
What is the most likely diagnosis in a patient with pancytopenia and Auer rods?
Acute promyelocytic leukemia.
448
What is the chromosomal translocation associated with acute promyelocytic leukemia?
t(15;17).
449
How can low-risk neutropenia with fever be managed?
It can be managed with ciprofloxacin and amoxicillin–clavulanate.
450
What should patients with sickle cell disease and moderate or severe acute chest syndrome receive?
They should receive short-term care consisting of red-cell exchange transfusion.
451
What test is used to confirm a suspected diagnosis of autoimmune hemolytic anemia?
A direct antiglobulin test.
452
What may ITP patients who relapse or are unresponsive to dexamethasone respond to?
They may respond to intravenous immunoglobulin stimulation of platelet production with eltrombopag or romiplostim.
453
What is the treatment for patients refractory to medical management?
Splenectomy is reserved for patients refractory to medical management.
454
What is a leading cause of death in adult patients with sickle cell disease?
Acute chest syndrome is a leading cause of death in adult patients with sickle cell disease.
455
When should positron emission tomography and electron microscopy be used?
They should be used only if the diagnosis is unclear after light microscopy and IHC.
456
What should patients with essential thrombocythemia and risk factors for complications be treated with?
They should be treated with cytoreductive therapy with hydroxyurea.
457
What does the 4Ts score for HIT take into account?
The 4Ts score takes into account the (1) degree of thrombocytopenia, (2) the timing of the decline in platelet count, (3) the development of thrombosis or other sequelae, and (4) the presence of other causes for thrombocytopenia.
458
What is squamous-cell carcinoma of the oropharynx associated with in non-smokers?
It is most often associated with human papillomavirus infection.
459
What forms a complex in the coagulation process?
Factor VIIa and tissue factor form a complex.
460
What is the mean age at diagnosis of renal-cell carcinoma in patients with von Hippel–Lindau disease?
The mean age at diagnosis is approximately 44 years.
461
What characterizes essential thrombocythemia?
It is characterized by excessive production of platelets in the absence of increased red-cell mass.
462
What is one of the minor diagnostic criteria for systemic mastocytosis?
A serum tryptase level >20 ng/mL in the absence of an associated myeloid disorder.
463
What is the recommended initial anticoagulant for uncomplicated venous thromboembolism?
The recommended initial anticoagulant is a DOAC.
464
What is a DOAC?
A DOAC is a direct oral anticoagulant that does not correct the activated partial-thromboplastin time in a mixing study, suggesting the patient’s plasma contains a factor that inhibits one of the clotting factors.
465
What is the most common factor inhibited in patients with a DOAC?
The most common factor inhibited is factor 8.
466
What treatment has been associated with significant improvements in overall survival among patients receiving carboplatin and paclitaxel for metastatic non-small-cell lung cancer?
Bevacizumab.
467
What is the most appropriate treatment for a patient with end-stage liver disease and low fibrinogen levels who is bleeding with hemoglobin greater than 7?
Cryoprecipitate.
468
What is the most appropriate diagnostic test for an older man with an easily palpable breast mass?
Core biopsy.
