Hematology/Oncology Flashcards
(108 cards)
1
Q
What is the most common clinical presentation of a lymphoid malignancy in adults?
A
painless enlargement of lymph nodes with no explanation or lymphadenopathy
2
Q
What type of cancer is caused by the Epstein-Barr virus?
A
Burkitt’s lymphoma
3
Q
What type of cancer is caused by Helicobacter pylori?
A
gastric MALT lymphoma
4
Q
What are the B-symptoms?
A
Signs of lymphoma
- unexplained fevers
- drenching night sweats
- weight loss of 10% of body weight in 6 months
5
Q
What is the significance of the following in the Ann Arbor Staging System?
- Stage I
- Stage II
- Stage III
- Stage IV
A
- Stage I
- 1 node involved
- Stage II
- >1 node, above diaphragm
- Stage III
- >1 node, below diaphragm
- Stage IV
- Diffuse involvement
6
Q
What are the cell markers for Follicular lymphomas?
A
- CD19
- CD20
- CD21
- Negative for CD5
7
Q
Do B cell or T cell lymphomas have a worse prognosis?
A
T cell lymphomas
8
Q
Which are the most aggressive non-Hodgkin’s lymphomas?
A
Burkitt’s lymphoma
Lymphoblastic lymphoma
9
Q
Mantle Cell Lymphoma
- Cell markers
- Chromosomal abnormality
A
- Markers
- CD19, 20, 5 positive
- CD23-negative (distinguishes from CLL)
- Chromosome
- t(11;14)
10
Q
Lymphoblastic Lymphoma
- What nodes are affected?
- Where can it spread?
A
- Nodes:
- mediastinal
- Spread:
- bone marrow
- leptomeninges (brain)
11
Q
Burkitt’s Lymphoma
- Organ involvement
- Characteristic cell pattern
- Translocation
A
- Tumor lysis syndrome at start of therapy
- Organ involvement
- bone marrow
- CNS
- Characteristic cell pattern
- Starry sky pattern
- Translocation
- t(8;14)
12
Q
What is the pathology and cytology of Nodular Sclerosing Hodgkin’s Disease?
A
- Path
- fibrous bands separate nodes into nodules
- affects mediastinal and supradiaphragmatic nodes
- Cytology
- lacunar type Reed-Sternberg cells
13
Q
Nodular-Lymphocyte-predominant Hodgkin’s Disease
- Cytology
A
Popcorn cells
(No reed-sternberg cells)
14
Q
What are the cell markers of Reed-Sternberg cells? What disease is signaled by the presence of these cells?
A
- Markers: CD30, CD15
- Diagnosis: Hodgkin’s Disease
15
Q
What is the disease?
Patient presents with a generalized pruritis and B symptoms with shortness of breath. There is painless enlargement of the lymph nodes of the neck. Chest radiography shows mediastinal and hilar adenopathy. RS cells were found.
A
Hodgkin Disease
16
Q
What are some factors associated with a bad prognosis in Hodgkin’s Disease?
A
High erythrocyte sedimentation rate
Bulky disease (mediastinal widening by >1/3 or nodal mass >10cm)
17
Q
Chronic Lymphocytic Leukemia
- Markers
- Cytology
- Symptoms
A
- Markers:
- CD19, 20, 21 positive (B cells)
- CD 5 and 23 positive (differentiates from Mantel Cell)
- Cytology
- Smudge cells
- Symptoms: Blood-related
- thrombocytopenia
- Hemolytic anemia
- Hypogammaglobulinemia
18
Q
What is the RAI staging system for CLL?
- 0
- I
- II
- III
- IV
A
- 0
- only increased white count
- I
- 1 w/ lymphadenopathy
- II
- 1 & 2 w/ splenomegaly
- III
- 1-3 w/ anemia
- IV
- 1-4 w/ thrombocytopenia
19
Q
What is the disease?
What is the disease?
- Cell Markers
- CD19, 20, 5 positive
- CD23-negative
A
Mantle Cell Lymphoma
20
Q
What is the disease?
What is the disease?
- Tumor lysis syndrome at start of therapy
- Organ involvement
- bone marrow
- CNS
- Characteristic cell pattern
- Starry sky pattern
A
Burkitt’s Lymphoma
21
Q
What is the disease?
What is the disease? Be specific.
- Path
- fibrous bands separate nodes into nodules
- affects mediastinal and supradiaphragmatic nodes
- Cytology
- lacunar type Reed-Sternberg cells
A
Nodular Sclerosing Hodgkin’s Disease
22
Q
What is the disease?
What is the disease? Be specific.
Cytology reveals only Popcorn cells
A
Nodular-Lymphocyte-predominant Hodgkin’s Disease
23
Q
What is the disease?
What is the disease?
- Markers:
- CD19, 20, 21 positive
- CD 5 and 23 positive
- Cytology
- Smudge cells
- Symptoms: Blood-related
- thrombocytopenia
- Hemolytic anemia
- Hypogammaglobulinemia
A
Chronic Lymphocytic Leukemia
24
Q
What is the disease?
Patient presents with bone lesions, splenomegaly, and pancytopenia. A bone marrow biopsy was attempted but the aspiration was dry due to fibrosis. Cells are TRAP-positive and CD5 negative.
A
Hairy Cell Leukemia
25
What is the disease?
Patient has no symptoms, but blood tests show less than 10% bone marrow plasma cells and M proteins. Lytic bone lesions are also present.
Monoclonal gammopathy of unknown significance (MGUS)
26
What is the disease?
Patient has no symptoms, but blood tests show M protein and marrow plasma cells 10-30%.
Smoldering Myeloma
27
What is the disease?
Patient presents with lytic bone lesions, hypercalcemia, plasma cytosis and Bence Jones Protein. Blood smear shows rouleaux formation.
Multiple Myeloma
28
Staging System for Multiple Myeloma
* Stage I
* B2M \< 3.5, albumin \> 3.5
* Stage II
* B2M \< 3.5, albumin \< 3.5
* Stage III
* B2M \> 3.5, albumin \< 3.5
Albumin should be high, B2M should be low (Normal)
29
What is the effect of Waldenstrom's Macroglobulinemia on the blood?
* Hyperviscosity syndrome
* epistaxis
* retinal hemorrhages, CHF
30
28 yo old male presents with 6 month history of pruritis. On exam he was found to have bilateral cervical lymphadenopathy and also splenomegaly. What is the possible diagnosis and what is the clinical stage.
Hodgkin's Disease
Stage III
31
64 y old female presents with fatigue , tiredness and bone pains. On exam she was found to be anemic, skeletal survey showed punched out lesions. Her possible diagnosis.
Multiple Myeloma
32
75 y old male on routine CBC was found to have increased WBC of 17,000 with 85%lymphocytes. He is completely asymptomatic, and has no lymphadenopathy or organomegaly. Hemoglobin and platelets are normal.
Stage 0 CLL
33
What are the indications and complications of granulocyte transfusion?
* IND:
* sepsis
* Complications:
* must be ABO compatible
* Allergy
* CMV
* Graft vs Host Disease
34
By how much does a unit of packed RBCs increase amount in blood?
1 gram
35
By how much does one unit of platelets increase amount in blood?
8,000
36
What are the indications for platelet infusion?
* Plts \< 10,000
* Plts \< 20,000 w/ bleeding
37
What are the components of fresh frozen plasma? What are some indications?
* Components:
* coagulation factors (proteins C and S)
* Plasma proteins (fibrinogen)
* Indications:
* Coag factor deficiency
* Overdose of coumadin
* coagulopathy (ex: liver disease)
38
What are the components of Cryoprecipitate? What are its indications?
* Components:
* Factors VIII, XIII
* vWF
* Fibrinogen
* IND:
* DIC
* Hemophilia A
* Afibrinogemia
* vWD
39
What Ab is responsible for acute intravascular transfusion reactions associated with ABO incompatibility?
IgM
40
Hemolytic Transfusion Rxn
* Mediated by which Ab
* Prevention
* Treatment
ABO incompatibility
* IgM
* Prevention:
* pretransfusion compatibility test
* Treatment:
* stop transfusion immediately
* Infuse fluids with diuretic use
* protect kidneys
41
Febrile Nonhemolytic Reactions
* Mediated by which Ab
* Cause
* Symptoms
* Prevention
* Treatment
* Mediated by IgG?
* Cause
* HLA or leukocyte Ag on donor WBCs that react w/ recipient Ab
* Symptoms
* fever, chills
* Prevention
* Leukopore
* Treatment
* Antipyretic (Tylenol)
42
Allergic Transfusion Reactions
* Mediated by which Ab
* Cause
* Symptoms
* Prevention
* Treatment
* Mediated by IgG?
* Cause
* Ab to plasma proteins of donor
* Symptoms
* Urticaria
* Erythema
* Dyspnea
* Prevention/ Treatment
* Antihistamine
43
Anaphylactic Reactions
* Mediated by which Ab
* Cause
* Symptoms
* Prevention
* Treatment
* Mediated by IgA
* Cause
* AgA antibodies in IgA deficient patients
* Symptoms
* No fever
* flushing
* Hypotension
* Cardiac arrythmia
* Laryngeal edema
* Prevention
* Give IgA deficient blood
* Treatment
* Epinephrine
44
Transfusion Related Lung Injury (TRALI)
* Mediated by which Ab
* Cause
* Symptoms
* Prevention
* Treatment
* Mediated by IgG?
* Cause
* Ab react to granulocytes
* Lungs fill with high protein fluid
* Symptoms
* Acute respiratory insufficiency
* Prevention
* Avoid blood from multiparous women and those with history of multiple transfusions
* Treatment
* Steroids
45
Circulatory Overload
* Cause
* Symptoms
* Prevention
* Cause
* transfused too rapidly (overwhelms cardiopulmonary system)
* Symptoms
* Dyspnea
* headache
* CHF
* Prevention
* Place patient in upright position and pace infusion
46
Delayed Hemolytic Transfusion Reaction (DHTR)
* Mediated by which Ab
* Cause
* Mediated by IgG
* Cause
* Previously exposed to RBC antigen and has low Ab titer until exposed again
* DAT (Direct antiglobulin test) negative at first, but becomes positive
47
Graft vs Host Disease
* Cause
* Symptoms
* Prevention
* Cause
* donor lymphocytes transfused into immunocompromised recipient
* Symptoms
* Pancytopenia after 1.5 weeks
* Prevention
* Irradiated Leukopore Blood (kill T cells)
48
Posttransfusion Purpura
* Cause
* Symptoms
* Treatment
* Cause
* Alloantibody against a high-incidence platelet Ag (P1A1)
* Common in multiparous women
* Symptoms
* Platelet count drops below 10,000
* Treatment
* Infuse platelets
49
What is the most common cause of anemia?
Iron deficiency
50
Patient presents with weakness, palpitations, nail spooning, and cravings for ice. A peripheral blood smear is shown below. Labs show decreased serum iron, increased TIBC, and low ferritin. What is the diagnosis? What category of anemia is this?
Hypochromic Microcytic Anemia caused by Iron Deficiency
51
Patient presents with hypercellular marrow and low reticulocyte count. Labs show High iron, low TIBC, and high ferritin. A peripheral blood smear is shown below. What is the disease? What are some causes? What is the Pathology?
Sideroblastic anemia
Hypochromic microcytic anemia
* Cause:
* Myelodysplastic syndrome (MDS)
* Alcohol abuse
* Path:
* abnormal incorporation of iron into heme
* Iron accumulates in mitochondria
* Ineffective erythropoiesis
52
Patient was diagnosed with multiple myeloma two months ago. Lab tests show low iron, low TIBC, high ferritin, and increased bone marrow storage of iron. What is the disease? What is the pathophysiology?
Anemia of chronic disease
normocytic, normochromic
* Path:
* abnormal iron utilization (absorbed but released into plasma slowly)
* impaired erythropoiesis
* decreased erythrocyte survival time
53
How does chronic renal disease cause anemia?
decreased erythropoietin production
54
What is the pathophysiology of beta thalassemia? Alpha thalassemia?
* Beta thalassemia
* loss of beta chain, increased Hgb A2 (instead of Hgb AB)
* Alpha thalassemia
* loss of alpha chain, increased Hgb B2
* Lab tests:
* High iron
* Low TIBC
* High Ferritin
55
What anemia is characterized by hypersegmented neutrophils?
Pernicious anemia
56
Which test result differentiates B12 deficiency and folate deficiency as the cause of anemia?
* Elevated homocysteine
* narrows it down to B12 or folate
* Elevated MMA
* B12 (pernicious anemia)
57
What are the differences between warm and cold autoimmune hemolytic anemias in regards to Ab, MOA, onset, jaundice, and splenomegaly?
* Warm
* IgG
* both IgG and complement mediated
* abrupt onset
* jaundice and splenomegaly present
* Cold
* IgM
* Complement only
* gradual onset
* no jaundice or splenomegaly
58
What are the associated diseases of warm and cold autoimmune hemolytic anemias?
* Warm
* Drugs
* Cold
* Mycoplasma infections
* Viral infections
59
What type of anemia is characterized by teardrop RBCs and nucleated RBCs in the periphery? What is the cause?
* Myelophthisic processes
* Marrow infiltration
* Myelofibrosis
60
Patient has pancytopenia, low reticulocyte count, and bone marrow biopsy shows all fat cells with very few blood cells. What is the disease?
Aplastic anemia
61
* 32 yo WF with heavy menstruation
* HGB 7.2
* MCV 71 (low)
* RDW 18 (high)
* Reticulocyte count \< 1.0%
* What’s the cause?
* How do you treat this patient?
Cause: iron deficiency
Treatment: iron supplement
62
* 58 yo WF with severe fatigue, memory problems, numbness/paresthesias in her toes
* Hgb 8.2 (low)
* WBC 3.5 (low)
* Plt 88 (low)
* MCV 124 (high)
* Reticulocyte count \< 1.0%
* What’s the diagnosis?
* How would you treat this patient?
Diagnosis: Vitamin B12 deficiency
Treatment: Vit B12 injections
63
* 48 yo AAF with fatigue, dark urine x 3 days, jaundice
* Hgb 5.5
* MCV 98
* WBC 35.0 with 90% lymphocytes
* Plt normal
* Reticulocyte count 15%
* LDH 350
* Haptoglobin \< 6
* Bilirubin 4.8 (4.0 indirect bilirubin)
* What else would you order and how would you treat?
* What other disease does this patient probably have?
Disorder: Autoimmune Hemolytic Anemia
Order: Coombs test
Treatment: Steroids
Other disease Pt could have: CLL
64
* 75 yo AAM with 10 year history of prostate cancer, failed hormonal therapy, new bone pain and fatigue
* Hgb 9.5
* MCV 88
* Retic 1.5%
* WBC/plt normal
* Smear shows many nRBC/teardrop RBC
* What’s the diagnosis?
Diagnosis: invasion of bone marrow by cancer cells (myelophthisic process)
65
* 54 yo asymptomatic middle eastern woman
* Hgb 10.9
* MCV 61
* RDW 12
* What test would you order next?
Hemoglobin electrophoresis
Beta-Thalasemia minor
66
* 65 yo WM with six month history of back pain
* Hgb 9.0
* RBC indices/WBC/plt normal
* Calcium level 14 (high)
* Creatinine 2.3 (high)
* Total protein 12.0 (high)
* What test would you order next?
* Does this patient need a BM biopsy?
Trick case
* Pt has Multiple myeloma
* hypercalcemia = bone lysis
* High creatinine = renal failure
* High protein = clonal Ig
67
* 43 yo WF with chronic rheumatoid arthritis
* Hgb 9.8
* MCV 88 (Normal)
* WBC/plt normal
* Reticulocyte count 1.5% (low)
* What would the iron studies look like?
* What is the diagnosis?
Iron studies: Low
Diagnosis: Anemia of chronic disease
68
What are the symptoms of SVC Syndrome? What is the cause?
Superior vena cava = SVC
* Symptoms:
* Dyspnea
* Neck and facial swelling
* Venous distention in the neck and chest wall
* Cause:
* elevated central venous pressure
69
How is SVC syndrome diagnosed?
Chest CT
* shows characteristics of venous flow and blockage
70
Which tumors types tend to metastasize to spinal column?
Breast
Prostate
Lung
71
What are the symptoms of Epidural Spinal Cord Compression (ESCC)? What is the treatment?
* Symptoms:
* pain
* motor weakness
* Treatment:
* Corticosteroids (decrease swelling)
* especially w/ brain compression
72
What are the symptoms of Acute Tumor Lysis Syndrome? In what cancer-type is it normally found?
Symptoms:
* Increased:
* (P) Phosphate
* (K) Potassium
* uric acid
* Decreased
* (Ca) calcium
* Oligouric renal failure
Found in Burkitt's lymphoma
73
What is the treatment plan and prevention for hyperuricemia and renal failure in ATLS?
* Prevention: Allopurinol
* inhibits xanthine oxidase
* inhibits uric acid formation
* Treatment:
* Rasburicase
74
What is the treatment for Hypercalcemia?
1. Hydration with normal saline
2. Loop diuretics
3. Bisphosphonates
* inhibit osteoclasts
* reduce bone resorption
75
What is the function of GP IIb-IIIa?
Fibrinogen receptor, platelet aggregation
76
What is the function of Gp Ib-IX-V?
von Willebrand factor receptor, initiate platelet adhesion to endothelium
77
What is in dense granules?
ADP and Ca2+, platelet aggregation
78
What is the function of Abciximab and Epibatidine? Indication?
* Function: inhibit GP IIb/IIIa
* IND: MI
79
What is the function of Plavix and Pasugrel? Indication?
* Function: Inhibits ADP and platelet aggregation
* IND: MI
80
Bernard Souilier Syndrome
* Pathophysiology
* Symptoms
* Path:
* **absence of Gp Ib-IX-V receptor**
* weak clotting
* Symptoms:
* minor bleeding
* bruise easily
81
Glanzmann's Thrombasthenia
* Pathophysiology
* Treatment
* Path:
* GP IIb/IIIa deficiency
* Treatement
* Platelet transfusions as needed
82
Von Willebrand Disease
* Type I
* Type II
* Type III
* Type I
* low protein
* Type II
* protein is present, but nonfunctional
* Type III
* No protein
83
Hemophilia
* Inheritance pattern
* A vs B
* Treatment
* Inheritance pattern
* X-linked
* A vs B
* A: Factor VIII deficient
* B: Factor IX deficient
* Treatment
* DDAVP
84
What is the function of protein C/ Protein S?
degrades Va and VIIIa
85
What does thrombin time measure?
Measures conversion of fibrinogen to fibrin
Measures dysfibrinogenemia or hypofibrinogenemia
86
What factors are assayed using PT?
VII, X, V, III, and II
87
What factors are assayed with PTT?
XII, XI, IX, VIII
88
Immune Thrombocytopenic Purpura
* What stimulates it?
* Path
* Treatment
* Preceded by viral infection in children
* Path
* Ab-mediated destruction of platelets by spleen (but normal production)
* Treatment:
* Thombopoeitin mimetic
89
Thrombotic Thrombocytopenic Purpura
* Pathophysiology
* Labs
* Treatment
* Path:
* deficiency of vWF-cleaving proteins
* HMW vWF
* increased platelet adhesion and clearance w/o activating coagulation cascade
* Labs
* fibrinogen normal, elevated LDH
* schistocytes present
* Treatment:
* Plasmapheresis, immunosuppression, antiplatelet drugs
90
Patient presents with bruising and abnormal bleeding. Labs show DIC panel is normal (fibrinogen is normal) and elevated LDH. Schistocytes are found in a peripheral smear. What is the disease?
TTP
91
What normally catalyzes Hemolytic Uremic Syndrome?
E. coli 0157.H7 serotype infection
Treat with plasmapheresis
symptoms similar to TTP
92
What is the cause of Atypical Hemolytic Uremic Syndrome?
Complement-mediated thrombotic microangiopathy (TMA)
93
What is the MOA of Soliris?
binds to C5 to block complement cascade (pore formation)
94
DIC
* Pathophysiology
* Labs
* Path
* trauma or infection causes tissue factor release
* generation of fibrin
* Not enough factors left to coagulate when needed
* Peripheral destruction of platelets
* Labs: Low fibrinogen level
95
Venous Thromboembolism
* Cause (genetic and other)
* Diagnosis
* Treatment
* Cause:
* Factor V Leiden Mutation
* APC resistance
* Antiphospholipid Ab/ Lupus anticoagulant
* Diagnosis
* D-Dimer assay
* If present, there is no active clot
* Treatment
* Anticoagulation therapy
96
Activation of BCR-ABL signals what disease?
CML
97
Patient presents with erythrocytosis, high hemoglobin and hematocrit, and aquagenic pruritis. Labs show low EPO and a JAK-2 mutation. What is the disease? What serious complications can arise?
* Disease: Polycythemia Vera
* Complications:
* hyperviscosity of the blood can cause venous or arterial thrombosis
* cerebral, cardiac, mesenteric most common (unusual sites)
* Portal vein thrombosis causes erythromelalgia
* erythema, burning, and pain in extremities
*
98
What is the cause of Primary PV?
JAK-2 V617F mutation
99
What are some causes of erythrocytosis?
* COPD
* Androgens
* PV
100
What are the criteria for Polycythemia vera diagnosis?
* Increased Hb
* JAK-2 mutation
* decreased EPO
101
What is the treatment of Polycythemia Vera?
* Phlebotomy
* Remove excess RBCs
* Aspirin
102
Patient presents with thrombocytosis. Has a history of easy bruising and thrombotic tendencies. Bone marrow biopsy shows increased numbers of enlarged, mature megakaryocytes. Labs show the patient is JAK-2 V617F positive. What is the disease?
Essential Thrombocythemia (ET)
Must prove it is not reactive
103
Patient presents with night sweats, fatigue, and weight loss. Examination shows splenic enlargement. Blood smears show nucleated RBCs and teardrop shaped RBCs. Bone marrow biopsy shows fibrosis. What is the disease?
Primary Myelofibrosis
104
What population is most affected by Primary Myelofibrosis?
men over 60
105
Patient presents with splenomegaly and leucocytosis with basophilia and thrombocytosis. Karyotyping shows a t(9;22) and patient is bcr-able (+). What is the disease? What disease always results from it?
* Disease: CML
* Result: Acute Leukemia
106
What are the treatment options for CML?
1. Allogenic stem cell transplant
2. Gleevec
* competitive inhibitor of bcr-abl
* mimics ATP
* delays progression to accelerated and blast phases and increases remissions
3. Dasatinib/ Nilotinib
* if resistant to Gleevec
107
Patient presents with anemia and abnormal bleeding. No splenomegaly or lymphadenopathy is present. Blood smear shows promyelocytes and cells with auer rods. What is the disease?
AML
Treatment: Chemo, marrow transplant, or both
108
Acute Promyelocytic Leukemia (APML)
* Characteristic findings
* Treatment
* Prognosis
* Characteristic findings
* promyelocytic cells with primary granules in cytoplasm
* granules: thrombogenic
* t(15;17)
* Treatment
* Tretinoin: targets cells with t(15;17)
* Arsenic trioxide if Tretinoin is ineffective
* Prognosis
* Good
* Determined by presence of t(15;17) cells after treatment
