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USMLE Step 2 CK Flash Cards > Hematology/Oncology > Flashcards

Hematology/Oncology Flashcards

1
Q

What do you do next when diagnosing megaloblastic anemia by vitamin B12 deficiency?

A

Confirm the etiology before treatment (better route is intramuscular)

*Dietary absence (vegans), pregnancy, malabsorption syndrome, ileal or gastric resection, pernicious anemia

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2
Q

History suggestive of pernicious anemia. How do you confirm the diagnosis?

A
  • Dyspepsia, autoimmune condition (ex, diabetes type 1), elderly
  • Low B12 levels
  • Confirm: Serum anti-intrinsic factor antibodies and anti-parietal cell antibodies
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3
Q

What is the therapy for Heparin-induced Thrombocytopenia (HIT)?

A
  • Stop Heparin (Unfractionated or LMW)
  • Continue anticoagulation with non-heparin medication (argatroban, fondaparinux)

*Lepirudin was discontinued

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4
Q

Most common cause of sepsis in sickle cell disease

A

Streptococcus pneumoniae→despite immunization, is due to non-vaccine serotypes

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5
Q

Tumors that cause approximately 75% of all malignant pleural effusions

A

Lung carcinoma, breast carcinoma and lymphoma

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6
Q

What is the cause of Febrile nonhemolytic transfusion reaction and how do you avoid it?

A
  • Cytokine release from leukocytes within the donor blood product
  • Leukoreduction of blood product

*Most common adverse transfusion reaction

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7
Q

Malignancies risks in a female patient with Lynch syndrome. Screening indication.

A
  • Colorectal cancer➡colonoscopy every 1–2 years starting at 25 years
  • Endometrial carcinoma➡annual endometrial biopsy beginning 30-35 years
  • Ovarian cancer

*If childbearing is complete, prophylactic hysterectomy and bilateral oophorectomy is recommended at 40 or earlier

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8
Q

Mechanism of jaundice in Vitamin B12 deficiency

A

Ineffective erythropoiesis▶⬆immature erythrocyte megaloblasts in bone marrow➡⬆intramedullary hemolysis➡⬆Heme▶⬆indirect bilirubin

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9
Q

Most common complication of Hemophilia A treatment with recombinant factor VIII

A

Inhibitor development➡immune system recognizes factor as foreign▶antibodies ❌factor

*⬆Bleeding frequency or hemorrhage refractory to treatment, ⬆PTT

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10
Q

Treatment for inhibitor development in a patient with Hemophilia A

A
  • Recombinant activated factor VII

- Activating prothrombin complex concentrates

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11
Q

Main manifestations of Von Hippel-Lindau disease

A
  • Hemangioblastoma in the retina and cerebellum (cystic nodular pattern)
  • Renal cell carcinoma (clear cell subtype, bilateral), preceded by multiple renal cysts (premalignant)
  • Pheochromocytoma

*Autosomal dominant

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12
Q

Hallmark of Paget’s disease and underlying breast condition

A
  • Painful, itching, eczematous, and/or ulcerating rash on the nipple that spreads to the areola
  • Adenocarcinoma
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13
Q

How do you confirm the diagnosis of Heparin-Induced Thrombocytopenia (HIT)?

A
  • Immunoassay➡⬆Titers

- Functional assay (Serotonin release assay) [Gold standard but less available]

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14
Q

Risk factors for splenic infarction

A
  • Hypercoagulable disorder
  • Source of embolic disease (atrial fibrillation)
  • Myeloproliferative neoplasm
  • Hemoglobinopathy (sickle cell disease)
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15
Q

Most common cause of megaloblastic anemia in chronic alcoholics

A

Folate deficiency

*Alcohol impair enterohepatic cycle and absorption of folate, may develop anemia in 5-10 wks (body stores are limited)

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16
Q

Effect of brussels sprouts, broccoli and green tea on warfarin

A

↓Effect→↑Thrombosis risk NO bleeding risk

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17
Q

Effect of acetaminophen on warfarin

A

↑Effect→↑Bleeding risk

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18
Q

Pathophysiology mechanism of Graft versus host disease

A

Activation of donor T-cells by recognition of host HLA-antigens▶cell-mediated immune response (Type IV hypersensitivity reaction)

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19
Q

Clinical presentation of chronic Graft versus host disease

A
  • Sicca syndrome
  • Chronic enteritis: Bloody diarrhea, Abdominal pain, Weight loss
  • Hepatic dysfunction: jaundice
  • Bronchiolitis obliterans: Chronic cough, Wheezing, Dyspnea
  • Myasthenic symptoms
  • Polymyositis
  • Scleroderma-like and lichenoid skin changes

*> 100 days after transplantation

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20
Q

When can you use anti-D immune globulin on Immune thrombocytopenia?

A

First-line treatment along with IVIG and corticoids in children if bleeding, and adults if bleeding or platelets≤30.000/mm3, and if Rh-positive and coombs negative→saturate Fc receptors of macrophages within the reticuloendothelial system (RES)►limiting the RES to clear platelets

*Do not give in Rh-negative patients

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21
Q

Cause of hemarthrosis with normal coagulation times and platelets in a patient with a history of bariatric surgery or restricted food intake

A

Vitamin C deficiency (scurvy)

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22
Q

Giant cell tumor of bone clinical presentation

A
  • Progressive knee pain + X-ray with eccentric lytic lesion (“soap bubbles”)
  • Benign, locally destructive neoplasm at epiphysis of long bones (young adults)
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23
Q

Giant cell tumor of bone association and prognosis

A
  • Associated with paget disease of bone

- It is benign but might have malignant transformation and pulmonary metastasis

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24
Q

Osteoid osteoma etiology and clinical presentation

A
  • Benign bone tumor prostaglandin-producer▶Nocturnal pain relieved by NSAIDs
  • X-ray: Small, round lucency
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25
Q

Clinical presentation of acute hemolytic transfusion reaction

A
  • Onset in minutes to 24 hours of transfusion
  • Fever, chills, hypotension
  • Renal tubular cell injury (may progress acute renal failure)▶Hemoglobinuria (dark urine), flank pain
  • Intravascular Hemolysis➡⬆LDH, ⬆Indirect bilirubin
  • DIC➡oozing intravenous site
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26
Q

Diagnosis test for acute hemolytic transfusion reaction

A

Direct coombs test➡antibody-coated RBCs

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27
Q

Primary dose-limiting side effect of first-line treatment for Sickle cell anemia with recurrent vaso-occlusive crisis

A

Myelosuppression (neutropenia, anemia, thrombocytopenia)

*Hydroxyurea➡⬆fetal hemoglobin➡⬇sickled hb➡⬇polymerization of RBC▶⬇vaso-occlusion episodes

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28
Q

Most common site of colon cancer metastasis

A

Liver

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29
Q

Musculoskeletal presentation of hereditary hemochromatosis

A

Arthralgia, arthropathy, chondrocalcinosis (Pseudogout)

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30
Q

How you may differentiate small cell carcinoma vs squamous cell carcinoma of the lung?

A

Paraneoplastic syndromes:

  • Small CC: ⬆ACTH (Cushing sx), ⬆ADH (SIADH), ⬆Antibodies against presynaptic Ca+2 channels (Lambert-Eaton myasthenic sx)
  • Squamous CC: ⬆PTHrP (hypercalcemia)
  • Sca++mous
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31
Q

Best treatment for cancer-related anorexia/cachexia syndrome (CACS)

A

Progesterone analogs (megestrol acetate medroxyprogesterone acetate)>corticosteroids

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32
Q

Which test you should order in an adult patient with isolated thrombocytopenia (Immune Thrombocytopenic Purpura)?

A

HIV and Hepatitis C➡most common secondary causes

*Initial presentation HIV up to 5-10%

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33
Q

Pathophysiology of Fanconi anemia

A

Autosomal recessive DNA repair defect, Bone-marrow failure

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34
Q

Clinical findings of Fanconi anemia

A
  • Short stature
  • Hypo or hyper-pigmented macula on trunk
  • Genitourinary malformations
  • Absence or hypoplastic thumb
  • Polydactyly, flat thenar eminence

*Most common cause of congenital aplastic anemia

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35
Q

Two most common inherited thrombophilia in the caucasian population

A
  1. Factor V Leiden (Activated Protein C resistance)

2. Prothrombin mutation (⬆Prothrombin levels)

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36
Q

When do you study or test for hereditary thrombophilias?

A
  • Young age (<45) with first time unprovoked DVT/PE
  • Recurrent DVT/PE
  • Unusual sited of thrombi (cerebral, mesentery, portal veins)
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37
Q

Treatment to reverse Warfarin toxicity bleeding

A

Prothrombin complex concentrate➡Vitamin K dependent coagulation factors (II, VII, IX, X, Protein C and S)

  • Room temperature
  • No transmit disease
  • No excess volume
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38
Q

Hemoglobin electrophoresis pattern of Beta-thalassemia major

A
  • HbA2 ⬆⬆ (2 alpha + 2 delta)
  • HbF ⬆⬆ (2 alpha + 2 gamma)
  • Mutation in both beta-globin genes
  • HbS absent, HbA absent
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39
Q

Treatment of Beta-thalassemia major

A

Transfusion-dependent➡⬆Risk of iron overload➡Iron chelation therapy▶avoid damage on liver, kidneys, endocrine glands

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40
Q

Findings on laryngoscopy of laryngeal squamous cell carcinoma in a patient with hoarseness

A

Fungating, irregular white/red mass on vocal cord with/without blood crusting (ulcer)

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41
Q

When do you suspect carcinoid syndrome?

A

Intermittent diarrhea + episodic flushing (85%) + cutaneous telangiectasias + bronchoespasm (wheezing) + cardiac abnormalities (right heart HF, tricuspid regurgitation)

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42
Q

Best initial diagnostic test for carcinoid syndrome

A

Urinary 5-hydroxyindolacetic acid (5-HIAA)

*Degradation product of Serotonin

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43
Q

Treatment for carcinoid syndrome

A

Octreotide

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44
Q

Most common benign cystic mass/tumor in the middle mediastinum

A

Bronchogenic cyst

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45
Q

Electrolyte abnormalities in Tumor lysis syndrome and their consequences

A
  • ↑Phosphorus
  • ↑Potassium→cardiac arrhythmia
  • ↑Uric acid→uric acid stones + AKI
  • ↓Calcium→calcium-phosphate stones + AKI

*Hypocalcemia is due to Hyperphosphatemia→↑calcium-phosphate precipitation (binding in the renal tubules)

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46
Q

What is the Her2/neu test and why is it done in breast cancer?

A
  • Abnormal estrogen receptor

- Positive➡anti-Her2/neu antibodies➡Trastuzumab▶⬇risk of recurrence and ⬆survival

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47
Q

Which is the hormonal manipulation or treatment for breast cancer? when are they indicated?

A
  • Tamoxifen or Raloxifene: premenopausal
  • Aromatase inhibitors (anastrozole, letrozole, exemestane): postmenopausal, slight superior in efficacy
  • All Estrogen or Progesterone Receptor patients: prevent recurrences
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48
Q

Most important adverse effects of tamoxifen and aromatase inhibitors

A
  • Tamoxifen➡Endometrial cancer and clots

- Aromatase inhibitor➡Osteoporosis

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49
Q

Available hormonal therapy in prostate cancer and for what is it used?

A
  • Flutamide, GNRH agonists, ketoconazole, orchiectomy➡help control the size and progression of metastases
  • Do not prevent recurrences; Do shrink lesions
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50
Q

Difference in the treatment of seminoma and non-seminoma testicular cancer

A
  • Seminomas: sensitive to chemotherapy and radiation

- Non-seminomas: sensitive to chemotherapy

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51
Q

Contraindication of 5HT inhibitors for chemotherapy-induced nausea

A
  • Ondansetron, granisetron, palonosetron, dolasetron: Best initial therapy
  • QT prolongation on EKG
52
Q

Biomarkers for testicular cancer

A
  • Alpha-fetoprotein: only for nonseminomatous cancers

- HCG: all testicular cancers

53
Q

What is the Abiraterone and for what do you use it?

A
  • Inhibitor of 17-hydroxylase➡❌synthesis of all androgens in the body (including adrenal)
  • ⬇Progression of metastatic prostate cancer and risk of death by 30%
54
Q

What is Pseudothrombocytopenia and its cause?

A
  • Laboratory error due to platelet aggregation in vitro
  • Incompletely mixed blood samples or serum antibodies to ethylenediaminetetraacetic acid (EDTA) (anticoagulant for hematologic testing)
55
Q

How may you confirm Pseudothrombocytopenia?

A
  • Peripheral blood smear➡large clumps of platelets

- Draw blood sample on non-EDTA anticoagulant tube (heparin, sodium citrate)➡normalizes platelet count

56
Q

Most common infections resulting from immunosuppression by solid organ transplantation

A

Pneumocystis pneumonia and Cytomegalovirus

57
Q

Clinical presentation and findings of Leydig cell tumor

A
  • Testicular mass
  • ⬆Estrogen➡gynecomastia, loss libido, erectile dysfunction
  • ⬆Testosterone➡acne, hirsutism

*Leydig cells➡Estrogen and testosterone source

58
Q

Expected findings of sexual hormones measurements on Leydig cell tumor

A
  • DO NOT produce B-hcg or AFP

- ⬆Estrogen and Testosterone➡⬇⬇FSH and LH

59
Q

Which is the only microcytic anemia that has high reticulocytes?

A

α-Thalassemia major or Hemoglobin H disease

60
Q

Finding on peripheral blood smear that suggests megaloblastic anemia, what is the most probable etiology?

A

Hypersegmented neutrophils➡only B12 and folate deficiency, and antimetabolite medications

*Many factors can cause macrocytic anemia, but not all hypersegmented neutrophils (Ex, Alcohol)

61
Q

Which tests may help to differentiate between B12 and folate deficiency? Why are they important when evaluating megaloblastic anemia?

A
  • B12 deficiency: ⬆Homocysteine, ⬆Methylmalonic acid (MMA)
  • Folate deficiency: ⬆Homocysteine, Normal MMA

*Remember! Both ⬆homocysteine; and in chronic or inflammatory (trauma, infection, cancer) conditions you might get normal B12 levels (⬆transcobalamin - acute phase reactant)➡get homocysteine and MMA even with normal B12 and folate levels if you have clinical suspicion

62
Q

Complication of B12 or folate replacement, why does it happen?

A

Extremely rapid cell production in the bone marrow packages up all the potassium➡Hypokalemia

63
Q

Why pancreatic insufficiency may cause megaloblastic anemia?

A

Pancreatic enzymes are needed to absorb B12➡remove B12 from the R-protein (carrier proteins)▶binds with intrinsic factor

64
Q

Why alcohol can cause sideroblastic anemia?

A

Suppressive effect on the bone marrow

*May cause macrocytic anemia as well

65
Q

How do you suspect an aplastic crisis in sickle cell disease? Which is the etiology?

A

Parvovirus B19➡First clue is a sudden drop in reticulocyte count

  • Almost always ⬆⬆Reticulocyte count in SCD
66
Q

Most accurate test for hereditary spherocytosis

A

Eosin-5 maleimide flow cytometry and acidified glycerol lysis test

67
Q

Most accurate test for warm antibody autoimmune hemolytic anemia

A

Direct Coombs test➡detects IgG (Warm) antibody on the SURFACE of the red blood cells

68
Q

Best initial treatment for warm antibody autoimmune hemolytic anemia. Second-line treatments if persist or more severe hemolysis.

A
  • Best initial: Glucocorticoids (Prednisone)
  • Second line:
    Rituximab (usually with prednisone as part of first-line)
    Intravenous immunoglobulin (IVIg)
    Azathioprine
    Cyclophosphamide
    Cyclosporine
69
Q

Most accurate test for cold agglutinin disease (cold antibody autoimmune hemolytic anemia)

A

Cold agglutinin (IgM) titer

*Direct coombs is positive only for complement

70
Q

Treatment for recurrent episodes of warm antibody autoimmune hemolytic anemia

A

Splenectomy

71
Q

Treatment for cold agglutinin disease

A
  • Keep the patient warm
  • Plasmapheresis
  • Rituximab

*Steroids and splenectomy do NOT work

72
Q

Best initial and most accurate test for Glucose 6 phosphate dehydrogenase deficiency

A
  • Peripheral blood smear➡Heinz bodies and bite cells

- Most accurate➡G6PD levels 1-2 months after an acute episode of hemolysis (is normal during or right after an episode)

73
Q

Most common cause of death or life-threatening complication of paroxysmal nocturnal hemoglobinuria

A

Thrombosis➡most common hepatic and mesenteric veins

*Secondary causes: infection due to severe neutropenia and hemorrhage due to severe thrombocytopenia

74
Q

Hematologic conditions associated with paroxysmal nocturnal hemoglobinuria

A
  • Previous history of (or may cause) Aplastic anemia or bone marrow failure
  • May cause Acute myeloid leukemia, myelofibrosis
75
Q

Classical features of Paroxysmal nocturnal hemoglobinuria clinical presentation

A
  • Episodic dark urine
  • Iron deficiency anemia
    Triad:
  • Pancytopenia
  • Hemolysis
  • Venous thrombosis (severe abdominal pain)
76
Q

Most accurate test for Paroxysmal nocturnal hemoglobinuria

A

CD55/CD59 absence via flow cytometry

77
Q

Best initial treatment for paroxysmal nocturnal hemoglobinuria

A

Prednisone for hemolysis

78
Q

Treatment for hemolysis and thrombosis in paroxysmal nocturnal hemoglobinuria

A

Eculizumab➡❌C5 in the complement pathway▶⬇Red blood cell destruction

79
Q

Most accurate test for polycythemia vera

A

JAK2 mutation

80
Q

Best therapy for polycythemia vera when hydroxyurea fails

A

Ruxolitinib➡inhibitor of JAK2

81
Q

Most accurate test for acute leukemia

A

Flow cytometry will distinguish the different subtypes of acute leukemia

82
Q

Indications of exchange transfusion in sickle cell disease

A

Severe vasoocclusive crisis:

  • Acute chest syndrome (pulmonary infarction, pneumonia)
  • Priapism
  • Stroke
  • Retinal infarction
83
Q

Treatments options for aplastic anemia besides supportive management

A
  • Allogenic bone marrow transplantation (BMT)➡young patients with a matched donor
  • Severe cases, no stem cell donor, or patients >50 yrs➡immunosuppress:
  • Cyclosporine➡❌T cells (Tacrolimus is alternative)
  • Antithymocyte globulin (ATG) and eltrombopag (prevent autoimmune marrow destruction)
  • Alemtuzumab➡anti-CD52▶❌T cells
84
Q

Treatment options for Myelofibrosis

A
  • Thalidomide, Lenalidomide➡TNF inhibitors▶⬆bone marrow production
  • <55 years➡attempt allogeneic bone marrow
    transplantation
  • Ruxolitinib➡❌JAK2▶suppresses myelofibrosis
85
Q

Which treatment you should add to M3 type acute myelogenous leukemia and why?

A

All-trans Retinoic Acid (ATRA)➡induces differentiation of promyelocytes

*t(15;17)➡PML gene; Retinoic acid receptor α▶Abnormal receptor

86
Q

What is a leukostasis reaction?

A
  • May happen in AML or ALL
    Blasts occluding the microcirculation➡pulmonary edema, CNS symptoms, ischemic injury, DIC

*⬆⬆WBC (>150,000/mm3 in AML, > 400,000/mm3 in ALL)➡⬆⬆Risk

87
Q

Treatment for leukostasis reaction

A
  1. Leukapheresis

2. Hydroxyurea

88
Q

Best initial therapy for chronic myelogenous leukemia

A

Tyrosine kinase inhibitors: Imatinib, Dasatinib, Nilotinib

*t(9:22)➡c-abl;bcr➡P210 protein▶tyrosine kinase activity

89
Q

Most effective cure for chronic myelogenous leukemia

A

Bone marrow transplantation

90
Q

Clinical presentation of acute leukemias

A

Bone marrow replaced by leukemia cells▶signs of pancytopenia (AML, ALL):

  • Anemia➡pallor, fatigue
  • ↓mature, ineffective, immature WBCs➡infections
  • Thrombocytopenia➡petechiae, purpura, bleeding
  • DIC most common in M3 (APL)
  • Medullary expansion into the periosteum➡bone pain (ALL)
  • Hepatosplenomegaly
91
Q

Most distinct laboratory anomaly in myelodysplastic syndrome. Which others you may find?

A

Pelger-Huet cells➡bilobed nucleus in neutrophils (“aviator glasses”)

  • Also:
  • Ringed sideroblasts
  • Nuclear budding
  • “Pawn ball” megakaryocytes
92
Q

Treatment for myelodysplastic syndrome

A
  • Transfusion➡support with blood products as needed
  • Erythropoietin➡20% response
  • Azacitidine or decitabine➡❌DNA methyltransferase➡hypomethylation of DNA (low dose), direct cytotoxicity to abnormal hematopoietic cells (high dose)
  • Lenalidomide➡for patients 5q deletion➡⬇transfusion dependence
  • Bone marrow transplant <50 years
93
Q

Which ultimate complication do acute lymphoblastic leukemia and chronic lymphocytic leukemia have in common?

A

Transformation to Lymphoma

  • ALL➡T-cell linage▶Lymphoblastic lymphoma
  • CLL➡Ritcher phenomenon or transformation (5% of patients)▶Small lymphocytic lymphoma (SLL)
94
Q

Which findings might suggest chronic lymphocytic leukemia?

A
  • Smudge cells
  • Hypogammaglobulinemia
  • AIHA or autoimmune thrombocytopenia
  • Lymphadenopathy
  • Infections

*SHALymfect

95
Q

Treatment for CLL

A

Depends on disease stage, usually III and IV

  • Fludarabine➡purine analog▶interfere with ribonucleotide reductase and DNA polymerase➡❌DNA synthesis
  • Cyclophosphamide➡Refractory cases or combination
  • Rituximab
  • Alemtuzumab➡anti-CD52 (when fludarabine fails)
  • Ibrutinib➡Bruton’s tyrosine kinase inhibitor
96
Q

Treatment for Non-Hodgkin lymphoma

A
  • Stage I/II➡Local radiation + small dose/course chemotherapy
  • Stage III/IV➡CHOP + Rituximab (R CHOP)

*CHOP: Cyclophosphamide, Doxorubicin (hydroxydaunorubicin), Vincristine (oncovin), Prednisone

97
Q

Treatment for Hodgkin lymphoma

A
  • Stage I/II➡Local radiation + small course/dose chemotherapy
  • Stage III/IV➡ABVD chemotherapy

*ABVD: Adriamycin (doxorubicin), Bleomycin, Vinblastine, Dacarbazine

98
Q

Most common presentation of multiple myeloma. Why does it happen?

A
  • Bone pain from pathologic fracture

- ⬆Osteoclast activating factor (OAF)➡lytic lesions➡hypercalcemia

99
Q

Most common causes of death in multiple myeloma

A

Renal failure and Infection

100
Q

How is the anion gap in multiple myeloma and why?

A

⬆IgG (Cationic)➡⬆⬆Cl-, HCO3- (anions)➡⬇Anion gap

101
Q

Most accurate test for multiple myeloma

A

Bone marrow biopsy➡>10% plasma cells

102
Q

How do you expect to find the plasma protein levels in multiple myeloma?

A

⬆Total protein with normal albumin

103
Q

Most common cause of IgG/A (M protein/spike) spikes on serum protein electrophoresis (SPEP). Which other causes you may think?

A
  1. Monoclonal gammopathy of unknown origin (MGUS)
  2. CLL, Lymphoma, Waldeström macroglobulinemia, amyloidosis

*Evaluate with bone marrow biopsy to exclude myeloma

104
Q

Best initial therapy for multiple myeloma

A

Dexamethasone + Lenalidomide or Bortezomib, or both

105
Q

Most effective therapy for multiple myeloma

A

If under 70, autologous bone marrow transplant with stem cell support

106
Q

Clinical presentation of Waldeström Macroglobulinemia

A
  • Hyperviscosity syndrome
  • Neurological deficits➡headache, vertigo, seizures, sensorimotor peripheral neuropathy, coma
  • Retinopathy➡Visual changes
  • Mucosal bleeding
  • Lethargy, weight loss, Raynaud phenomenon (cryoglobulinemia), engorged blood vessels on eyes (“sausage-link”: dilated, segmented, tortuous)
107
Q

Most accurate test for Waldeström Macroglobulinemia and its finding

A

Bone marrow biopsy➡Abnormal plasma cells with Dutcher bodies▶PAS⊕ IgM deposits around the nucleus

108
Q

Best initial therapy for Waldeström Macrobulinemia

A

Plasmapheresis➡removes excess IgM➡⬇viscosity

109
Q

Long term treatment for Waldeström Macrobulinemia

A
  • Rituximab or prednisone + cyclophosphamide

- Bortezomib or Lenalidomide

110
Q

Best initial test for Von Willebrand disease

A
  • ⬆Bleeding time in all vWD types, ⬆PTT (as in hemophilia, by ⬇factor VIII), normal PT and platelets
  • ⬇vWF antigen may be present
111
Q

Most accurate tests for Von Willebrand disease

A

Ristocetin cofactor assay➡⬇Agglutination

*Measures the capacity of vWF to agglutinate platelets and detects vWF dysfunction

112
Q

Best initial therapy for Von Willebrand disease and Hemophilia if moderate or mild severity

A

Desmopressin➡releases subendothelial stores of vWF and Factor VIIIa

*Factor level >5% of normal (mild) or 1–5% of normal (moderate)

113
Q

What do the hemophilias have in common regarding test results?

A

PT is normal, prolonged PTT➡Mixing studies with normal plasma will correct PTT (Best initial test)

114
Q

How do you confirm Heparin-induced thrombocytopenia?

A

ELISA for platelet factor 4 (PF4) antibodies

115
Q

Why Acute promyelocytic leukemia is considered an emergency?

A

⬆⬆Risk of pulmonary/cerebrovascular hemorrhage or blood-clotting problems from tumor-induced consumptive coagulopathy

*➕Tissue factor (DIC) + ⬆Plasmin (primary hyperfibrinolysis)

116
Q

Treatment for acute lymphoblastic leukemia M3 (APL)

A

Prompt treatment with All-trans retinoic acid (ATRA)➡⬆differentiation of promyelocytes▶⬇bleeding risk

*t(15;17)➡fusion PML gene + retinoic acid receptor alpha gene

117
Q

Clinical presentation of HUS and TTP. How you may differentiate them?

A
  • Microangiopathic hemolytic anemia (schistocytes, helmet cells, fragmented red blood cells)
  • Renal insufficiency
  • Thrombocytopenia
  • TTP
  • Neurological symptoms➡delirium, seizure, stroke, ↓consciousness, ↓vision
  • Fever
  • HUS
  • Hemorrhagic diarrhea preceding the syndrome (eating undercooked contaminated meat)
118
Q

Treatment of HUS and TTP

A
  • HUS from E. coli will resolve spontaneously
  • Plasmapheresis or Fresh frozen plasma for TTP and severe HUS
  • Steroids help in TTP, but NOT in HUS
119
Q

What is Atypical HUS and its treatment?

A
  • HUS does not arise from infection; complement erroneously attacks red blood cells
  • Eculizumab➡❌complement (C5a)
120
Q

Clinical presentation of acute Graft versus host disease

A
  • Maculopapular rash (palms, soles, face, generalized; painful, pruritic, confluent, Steven-Johnson syndrome-like)
  • Gastrointestinal: Profuse diarrhea (mucoid or watery, green or bloody), crampy abdominal pain, nausea, vomiting
  • Liver inflammation (damage biliary tract epithelium)➡⬆Bilirubin, AP, transaminases; Hepatosplenomegaly
  • In rare cases: lagophthalmos, hemorrhagic conjunctivitis, conjunctival pseudomembrane formation

*Within 100 days after hematopoietic stem cell transplantation

121
Q

Pathophysiology of acute graft versus host disease

A

Donor T-cells (cytotoxic) identify antigens on host epithelial cells as foreign➡strong proinflammatory response

122
Q

Most accurate test for porphyria cutanea tarda. What is the cause?

A
  • ⬆Uroporphyrin in a 24-hour urine collection

- Deficiency of Uroporphyrin decarboxylase activity

123
Q

Management of malignant pericardial effusion

A
  1. Acute drainage➡Pericardiocentesis
  2. Prevention of reaccumulation➡Pericardial window* or prolonged pericardial catheter drainage

Pericardiectomy to allow pericardial fluid to drain into pleural or peritoneal cavity

124
Q

What would be the most likely cause of kidney injury in an adult patient with hypercalcemia (progressive fatigue, muscle weakness), weight loss, and normocytic anemia?

A

Multiple myeloma

  • Monoclonal light chains clog renal tubules▶Myeloma cast nephropathy➡Renal tubular damage (normal urinalysis)
  • Amyloidosis and monoclonal immunoglobulin deposition disease➡glomerular injury and nephrotic syndrome (hematuria and proteinuria in urinalysis)
125
Q

Why does hydroxyurea cause macrocytic anemia?

A

Inhibits ribonucleoside reductase (important for DNA synthesis and repair)➡inhibits erythrocyte nuclear maturation and increases nuclear/cytoplasmic ratio➡⬆MCV

126
Q

Most likely cause of a patient with hearing loss and tinnitus after starting platinum-based chemotherapy

A
  • Medication side effect
  • Ototoxicity by cisplatin or carboplatin➡Irreversible, bilateral, high-frequency sensorineural hearing loss, tinnitus and/or imbalance

*Neurotoxic effects as well - peripheral neuropathy, CNS toxicity

USMLE Step 2 CK Flash Cards (17 decks)

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