Step 1 Express 2019-Hematology and Oncology

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First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY page 1

Hematology and Oncology


Questions

EMBRYOLOGY
1. Why are Rh-negative mothers given anti-D IgG? (p 397) _________________________________

______________________________________________________________________________

ANATOMY
2. What conditions can cause hypersegmentation of neutrophils? (p 398) ______________________

______________________________________________________________________________

3. Define the following terms. (p 399)

A. Anisocytosis _______________________________________________________________

B. Poikilocytosis ______________________________________________________________

C. Reticulocyte _______________________________________________________________

4. What do the dense granules of platelets contain? (p 399) ________________________________

5. What do the α-granules of platelets contain? (p 399) ____________________________________

6. CD14 is a cell surface marker for which cell type? (p 399) ________________________________

7. What seven conditions can cause eosinophilia? (p 400) __________________________________

______________________________________________________________________________

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page 2 First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY

8. What do basophilic granules contain? Which molecules are synthesized and released by basophils?
(p 400) ________________________________________________________________________

______________________________________________________________________________

9. B cells originate in the _______________ (bone marrow/thymus) and mature in the


_______________ (bone marrow/thymus). T cells originate in the _______________ (bone
marrow/thymus) and mature in the _______________ (bone marrow/thymus). (p 401)

10. What type of cell has an eccentric nucleus, abundant RER, and a clock-face chromatin

distribution? (p 401) ___________________________________________________________

11. Which lymphocytes are larger than B and T cells and can distinguish between healthy and infected

cells by identifying cell surface proteins? (p 401) _______________________________________

PHYSIOLOGY
12. Describe the four steps of platelet plug formation (primary hemostasis). (p 403)
______________________________________________________________________________

______________________________________________________________________________

______________________________________________________________________________

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First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY page 3

13. In the chart below, check the pathway(s) for each coagulation factor. (p 404)

Factor Extrinsic Pathway Intrinsic Pathway Both Pathways

II

VII

VIII

IX

XI

XII

14. Which factors are vitamin K dependent? How does warfarin inhibit the activation of these factors?

(p 405) _______________________________________________________________________

______________________________________________________________________________

PATHOLOGY
15. Identify each cell type and its associated pathology. (pp 406-408)

A. B. C. D. E. F.

A. _______________________________ D. ______________________________

B. _______________________________ E. ______________________________

C. _______________________________ F. ______________________________

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page 4 First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY

16. In α-thalassemia, what is the condition called when all four α-globin genes are deleted? When three
are deleted? When two are deleted? When one is deleted? (p 410) ________________________

______________________________________________________________________________

17. What clinical manifestations do folate deficiency and vitamin B12 deficiency have in common? What

sets them apart? (p 412) __________________________________________________________

______________________________________________________________________________

18. What are four causes of aplastic anemia? (p 413) ______________________________________

______________________________________________________________________________

19. Match the intrinsic hemolytic normocytic anemia with its characteristic. (p 414)

_____ A. G6PD deficiency 1. GLU → LYS mutation

_____ B. HbC disease 2. HbS point (GLU → VAL) mutation

_____ C. Hereditary spherocytosis 3. Heinz bodies and bite cells

_____ D. Paroxysmal nocturnal hemoglobinuria 4. Increased incidence of acute leukemias

_____ E. Pyruvate kinase deficiency 5. Increased fragility in osmotic fragility test

_____ F. Sickle cell anemia 6. Rigid RBCs

20. Which autoimmune hemolytic anemias are warm and which are cold? (p 415) ________________

______________________________________________________________________________

______________________________________________________________________________

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First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY page 5

21. Indicate whether the iron studies in the chart are elevated, decreased, or normal. (p 416)

Chronic Iron Pregnancy/ Hemo-


Lab Value
Disease Deficiency OCP use chromatosis

Ferritin

Serum iron

Transferrin or
TIBC

% Transferrin
saturation

22. In lead poisoning what enzymes are affected and what substrates are accumulated? (p 417) ____

______________________________________________________________________________

23. What are the “5 P’s” of acute intermittent porphyria? (p 417) ______________________________

______________________________________________________________________________

______________________________________________________________________________

24. Indicate whether the lab findings for the coagulation or platelet disorders in the chart are elevated,
decreased, or normal. (pp 418-419)

Platelet Bleeding
Disorder PT PTT
Count Time

Disseminated Intravascular
Coagulation (DIC)

Glanzmann thrombasthenia normal normal

Hemophilia A, B, or C normal normal

Thrombotic thrombocytopenic
purpura (TTP)
Vitamin K deficiency normal

von Willebrand disease

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page 6 First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY

25. Which type of Hodgkin lymphoma is most common? (p 421) _____________________________

______________________________________________________________________________

26. What type of cell is shown by the arrow in the image?


In what condition is this cell seen? (p 421)

________________________________________

________________________________________

________________________________________

27. What will serum protein electrophoresis reveal in a patient with multiple myeloma? What will be seen
in the urine? (p 423) ______________________________________________________________

______________________________________________________________________________

28. What are the four major groups of leukemia? Which type is at risk for DIC upon initiation of treatment
and why? (p 424) ________________________________________________________________

_______________________________________________________________________________

_______________________________________________________________________________

29. Match the disease with the genetic translocation most closely associated with it. (p 424, 426)

_____ A. Acute myelogenous leukemia 1. t(8;14)

_____ B. Burkitt lymphoma 2. t(9;22)

_____ C. Chronic myelogenous leukemia 3. t(11;14)

_____ D. Follicular lymphoma 4. t(14;18)

_____ E. Mantle cell lymphoma 5. t(15;17)

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First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY page 7

30. Indicate whether the lab findings in the chart are elevated, decreased, or normal. (p 425)

Chronic
Platelets RBCs WBCs
Myeloproliferative
Disorder
CML

Essential
thrombocythemia
Myelofibrosis

Polycythemia vera

PHARMACOLOGY
31. A patient presents with fever, pancytopenia, and hepatosplenomegaly. The bone marrow biopsy
shows macrophages phagocytosing marrow elements. What is the likely diagnosis? (p 427) _____

______________________________________________________________________________

32. What is the mechanism of action of heparin? How is overdose treated? (p 427) _______________

______________________________________________________________________________

33. What is the mechanism of action of warfarin? How is overdose treated? (p 428) ______________

______________________________________________________________________________

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page 8 First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY

34. Match each drug with its target. (pp 429-430, 432-435)

_____ A. Abciximab 1. ADP (P2Y12) receptor

_____ B. Clopidogrel 2. B cells (CD20)

_____ C. Etoposide 3. tyrosine kinase inhibitor of bcr-abl

_____ D. 5-Fluorouracil 4. Estrogen receptor

_____ E. Imatinib 5. Glycoprotein receptor IIb/IIIa

_____ F. Rituximab 6. HER-2 (c-erbB2)

_____ G. Tamoxifen 7. Plasminogen

_____ H. tPA 8. Thymidylate synthase

_____ I. Trastuzumab 9. Topoisomerase II

_____ J. Vincristine 10. β-tubulin

35. Match the patient with the drug he or she is most likely taking. (pp 431-433)

_____ A. Patient with previous bone marrow transplantation has 1. Bleomycin

PFTs consistent with restrictive lung disease 2. Busulfan

_____ B. Patient with colon cancer has myelosuppression 3. Cyclophosphamide

not reversible with leucovorin 4. Doxorubicin

_____ C. Patient with leukemia has myelosuppression, 5. 5-Fluorouracil

reversible with leucovorin 6. Methotrexate

_____ D. Patient with non-Hodgkin lymphoma has hemorrhagic

cystitis

_____ E. Patient with solid tumor has dilated cardiomyopathy

_____ F. Patient with testicular cancer has PFTs consistent with restrictive lung disease

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First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY page 9

Answers

EMBRYOLOGY
1. To prevent anti-D IgG formation, which can cause hemolytic disease of the newborn (erythroblastosis
fetalis) in a subsequent fetus.

ANATOMY
2. Vitamin B12 and folate deficiency.

3. A. Anisocytosis: Cells vary in size.

B. Poikilocytosis: Cells vary in shape.

C. Reticulocyte: Immature RBCs; reflects erythroid proliferation.

4. ADP and calcium.

5. von Willebrand factor (vWF), fibrinogen, fibronectin, and platelet factor 4.

6. Macrophages.

7. Parasites, asthma, Churg-Strauss syndrome, chronic adrenal insufficiency, myeloproliferative


disorders, allergic processes, and neoplasia (eg, Hodgkin lymphoma). Remember PACCMAN.

8. Basophilic granules contain histamine and heparin. They synthesize and release leukotrienes.

9. Bone marrow; bone marrow; bone marrow; thymus.

10. Plasma cell.

11. Natural killer cells.

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page 10 First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY

PHYSIOLOGY
12. 1. Injury: Endothelial damage occurs.

2. Exposure: vWF binds to exposed collagen.

3. Adhesion: Platelets bind vWF via the GpIb receptor at the site of injury and release ADP/Ca2+,
TXA2 ADP helps platelets adhere to endothelium.

4A. Activation: ADP binding to P2Y12 receptor induces GpIIb/IIIa expression at platelet surface.

4B. Aggregation: Fibrinogen binds GpIIb/IIIa receptors and links platelets.

13.

Factor Extrinsic Pathway Intrinsic Pathway Both Pathways

I √

II √

V √

VII √

VIII √

IX √

X √

XI √

XII √

14. Factors II, VII, IX, X, and proteins C and S. Warfarin inhibits vitamin K epoxide reductase, which is
necessary to convert vitamin K to its reduced form so that it can activate these factors.

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First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY page 11

PATHOLOGY
15. A = Degmacyte (bite cell): G6PD deficiency. B = Ringed sideroblast: sideroblastic anemia.
C = Schistocyte: microangiopathic hemolytic anemias (including DIC, TTP/HUS, HELLP syndrome,
mechanical hemolysis). D = Dacrocyte (teardrop cell): bone marrow infiltration (eg myelofibrosis),
thalassemias. E = Target cell: HbC disease, asplenia, liver disease, thalassemia.
F = Heinz bodies: G6PD deficiency.

16. Four deletions: Hemoglobin Barts disease; three deletions: Hemoglobin H disease (HbH); two
deletions: α-thalassemia minor; one deletion: α-thalassemia minima.

17. Both folic acid and vitamin B12 deficiency can cause megaloblastic anemia. Only vitamin B12
deficiency is associated with neurologic symptoms, such as reversible dementia and subacute
combined degeneration. B12 deficiency is also associated with increased methylmalonic acid, unlike
folic acid deficiency.

18. Idiopathic, radiation and drug exposure (eg, benzene, chloramphenicol), viral agents (EBV, HIV,
hepatitis viruses), and Fanconi anemia.

19. A-3, B-1, C-5, D-4, E-6, F-2.

20. Warm (IgG): chronic anemia seen in SLE and chronic lymphocytic leukemia (CLL) with certain
drugs.
Cold (IgM and complement): acute anemia triggered by cold; seen in chronic lymphocytic
leukemia, Mycoplasma pneumoniae infections, and infectious mononucleosis.

21.

Chronic Iron Pregnancy/ Hemo-


Lab Value
Disease Deficiency OCP use chromatosis
Ferritin ↑ ↓ normal ↑

Serum iron ↓ ↓ normal ↑

Transferrin or ↓ ↑ ↑ ↓
TIBC

% Transferrin normal ↓↓ ↓ ↑↑
saturation

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page 12 First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY

22. Enzymes: Ferrochelatase and ALA dehydratase. Substrates: Protoporphyrin and ALA (blood)

23. Painful abdomen, Port wine-colored urine, Polyneuropathy, Psychological disturbances, and
Precipitated by drugs (eg, cytochrome P-450 inducers), alcohol, and starvation.

24.

Platelet Bleeding
Disorder PT PTT
Count Time
Disseminated Intravascular ↓ ↑ ↑ ↑
Coagulation (DIC)
Glanzmann thrombasthenia normal ↑ normal normal

Hemophilia A, B, or C normal normal normal ↑

Thrombotic thrombocytopenic ↓ ↑ normal normal


purpura (TTP)
Vitamin K deficiency normal normal ↑ ↑

von Willebrand disease normal ↑ normal normal / ↑

25. Nodular sclerosis.

26. Reed-Sternberg cell; Hodgkin lymphoma.

27. An M-spike, representing a monoclonal antibody; Ig light chains in urine (Bence Jones proteins).

28. AML, ALL, CML, and CLL. AML can be associated with DIC upon initiation of treatment because the
leukemia cells contain Auer rods composed of the enzyme peroxidase, which is released into the
bloodstream when treatment causes cells to lyse.

29. A-5, B-1, C-2, D-4, E-3.

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First Aid Express 2019 workbook: HEMATOLOGY & ONCOLOGY page 13

30.

Chronic
Platelets RBCs WBCs
Myeloproliferative
Disorder
CML ↑ ↓ ↑

Essential ↑ — —
thrombocythemia
Myelofibrosis variable ↓ variable

Polycythemia vera ↑ ↑ ↑

PHARMACOLOGY

31. Hemophagocytic lymphohistiocytosis, which is a systemic overactivation of macrophages and


cytotoxic T cells.

32. Heparin activates antithrombin, decreasing action of thrombin and factor Xa. Treat heparin overdose
with protamine sulfate.

33. Warfarin inhibits epoxide reductase, which interferes with γ-carboxylation of vitamin K-dependent
clotting factors II, VII, IX, X, and proteins C, and S. Treat warfarin overdose with vitamin K; for rapid
reversal, treat with fresh frozen plasma, or PCC.

34. A-5, B-1, C-9, D-8, E-3, F-2, G-4, H-7, I-6, J-10.

35. A-2, B-5, C-6, D-3, E-4, F-1.

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