MD Hematology and Oncology 3 — Questions and Answers
Question 1: A 25-year-old man with hemophilia A requires surgery. His factor VIII level is 2%. Which product replaces the deficient factor and is the treatment of choice?
- Fresh frozen plasma
- Cryoprecipitate
- Recombinant factor VIII concentrate (Correct answer)
- Desmopressin (DDAVP)
Correct answer: Recombinant factor VIII concentrate
Recombinant factor VIII concentrate is first-line for severe hemophilia A; DDAVP is only effective for mild hemophilia A (factor VIII >5%).
Question 2: A 60-year-old man has lymphadenopathy, hepatosplenomegaly, WBC 80,000/μL with 95% mature small lymphocytes, and positive CD5/CD23 by flow cytometry. What is the diagnosis?
- Mantle cell lymphoma
- Chronic lymphocytic leukemia (Correct answer)
- Hairy cell leukemia
- Follicular lymphoma
Correct answer: Chronic lymphocytic leukemia
CLL is characterized by clonal proliferation of mature CD5+/CD23+ B lymphocytes with absolute lymphocytosis; mantle cell lymphoma is CD5+ but CD23−.
Question 3: An 8-year-old child presents with pallor, jaundice, and splenomegaly. Peripheral smear shows spherocytes and reticulocytosis. The osmotic fragility test is positive. What is the inheritance pattern?
- X-linked recessive
- Autosomal recessive
- Autosomal dominant (Correct answer)
- Mitochondrial
Correct answer: Autosomal dominant
Hereditary spherocytosis is most commonly autosomal dominant (spectrin or ankyrin mutations), though autosomal recessive forms exist; autosomal dominant accounts for ~75% of cases.
Question 4: Which staging system is used for Hodgkin lymphoma and defines Stage III as involvement of lymph node regions on both sides of the diaphragm?
- Ann Arbor staging (Correct answer)
- Rai staging
- Binet staging
- FLIPI scoring
Correct answer: Ann Arbor staging
The Ann Arbor staging system (with Cotswolds modification) classifies Hodgkin and non-Hodgkin lymphomas; Stage III involves nodes on both sides of the diaphragm.
Question 5: A patient with multiple myeloma shows a serum M-spike on SPEP. Which immunoglobulin heavy chain is most commonly involved in multiple myeloma?
- IgA
- IgG (Correct answer)
- IgM
- IgD
Correct answer: IgG
IgG myeloma is the most common subtype, accounting for approximately 50–60% of multiple myeloma cases.
Question 6: A 40-year-old woman develops microangiopathic hemolytic anemia, thrombocytopenia, fever, renal failure, and neurological symptoms. ADAMTS13 activity is <5%. What is the diagnosis?
- Hemolytic uremic syndrome
- Thrombotic thrombocytopenic purpura (Correct answer)
- DIC
- Evans syndrome
Correct answer: Thrombotic thrombocytopenic purpura
TTP is caused by ADAMTS13 deficiency (activity <10%), leading to ultra-large vWF multimers and platelet microthrombi causing the pentad of features.
Question 7: A patient with acute leukemia develops a prolonged PT, aPTT, low fibrinogen, elevated D-dimer, and thrombocytopenia. Which leukemia subtype is classically associated with this coagulopathy?
- AML-M2 (with t(8;21))
- AML-M3 (APL with t(15;17)) (Correct answer)
- ALL (B-cell)
- CML blast crisis
Correct answer: AML-M3 (APL with t(15;17))
Acute promyelocytic leukemia (APL/AML-M3) releases procoagulants from granules causing DIC; ATRA is urgently needed before cytotoxic chemotherapy.
A 25-year-old man with hemophilia A requires surgery.
His factor VIII level is 2%.
Which product replaces the deficient factor and is the treatment of choice?