MD Hematology and Oncology 5 — Questions and Answers
Question 1: A 22-year-old woman has a lifelong history of heavy menstrual bleeding, prolonged bleeding after dental procedures, and a normal PT and platelet count but prolonged aPTT and PFA-100 closure time. von Willebrand factor antigen is 35% (low). What is the most likely diagnosis?
- Hemophilia A
- Type 1 von Willebrand disease (Correct answer)
- Bernard-Soulier syndrome
- Glanzmann thrombasthenia
Correct answer: Type 1 von Willebrand disease
Type 1 VWD is the most common inherited bleeding disorder, characterized by quantitative reduction of VWF causing mucocutaneous bleeding with prolonged PFA-100 and aPTT.
Question 2: A patient with NHL undergoing CHOP chemotherapy develops neutropenic fever on day 10. ANC is 200/μL. What is the most appropriate initial empiric antibiotic choice?
- Vancomycin + metronidazole
- Antipseudomonal beta-lactam (e.g., piperacillin-tazobactam) (Correct answer)
- Oral ciprofloxacin monotherapy
- Caspofungin alone
Correct answer: Antipseudomonal beta-lactam (e.g., piperacillin-tazobactam)
IDSA guidelines recommend empiric broad-spectrum antipseudomonal beta-lactam monotherapy as initial treatment for febrile neutropenia.
Question 3: On peripheral smear, teardrop cells (dacrocytes), nucleated RBCs, and immature granulocytes are seen together. This leukoerythroblastic picture is classic for:
- G6PD hemolysis
- Myelophthisic anemia / myelofibrosis (Correct answer)
- Megaloblastic anemia
- Autoimmune hemolytic anemia
Correct answer: Myelophthisic anemia / myelofibrosis
Myelofibrosis or bone marrow infiltration (myelophthisis) causes a leukoerythroblastic smear with teardrop cells as RBCs are squeezed through fibrotic marrow.
Question 4: A 55-year-old man has an absolute eosinophil count of 6,000/μL for >6 months with evidence of cardiac damage. FISH reveals FIP1L1-PDGFRA fusion. First-line treatment is:
- Hydroxyurea
- Imatinib (Correct answer)
- Mepolizumab
- High-dose corticosteroids
Correct answer: Imatinib
FIP1L1-PDGFRA-positive hypereosinophilic syndrome responds dramatically to imatinib (a PDGFR kinase inhibitor), achieving complete remission in most cases.
Question 5: A 70-year-old man has IgM paraprotein of 4 g/dL, lymphadenopathy, bone marrow infiltration by lymphoplasmacytic cells, and hyperviscosity symptoms. What is the diagnosis?
- Multiple myeloma
- Waldenström macroglobulinemia (Correct answer)
- MGUS
- Marginal zone lymphoma
Correct answer: Waldenström macroglobulinemia
Waldenström macroglobulinemia is defined by IgM monoclonal protein plus bone marrow infiltration by lymphoplasmacytic lymphoma, commonly causing hyperviscosity.
Question 6: A 45-year-old woman undergoes allogeneic stem cell transplant. On day 35, she develops diarrhea, skin rash, and rising bilirubin. What is the most likely complication?
- Graft failure
- Acute graft-versus-host disease (Correct answer)
- Veno-occlusive disease
- CMV colitis
Correct answer: Acute graft-versus-host disease
Acute GVHD (typically within 100 days post-transplant) classically affects skin (rash), gut (diarrhea), and liver (hyperbilirubinemia) as donor T cells attack host tissues.
Question 7: A 32-year-old man presents with painless cervical lymphadenopathy. Biopsy reveals Reed-Sternberg cells with owl-eye nucleoli in a background of lymphocytes. Which immunophenotype is characteristic of these cells?
- CD20+, CD3−
- CD15+, CD30+, CD45− (Correct answer)
- CD5+, CD23+
- CD10+, BCL2+
Correct answer: CD15+, CD30+, CD45−
Reed-Sternberg cells in classical Hodgkin lymphoma are CD15+/CD30+ and characteristically CD45− (leukocyte common antigen negative).
MD Hematology and Oncology 5
A 22-year-old woman has a lifelong history of heavy menstrual bleeding, prolonged bleeding after dental procedures, and a normal PT and platelet count but prolonged aPTT and PFA-100 closure time. von Willebrand factor antigen is 35% (low).
What is the most likely diagnosis?