AOCNP Hematologic Malignancies 2 — Questions and Answers
Question 1: Reed-Sternberg cells, large binucleated cells with prominent 'owl-eye' nucleoli, are pathognomonic of which disease?
- Diffuse large B-cell lymphoma (DLBCL)
- Classical Hodgkin lymphoma (Correct answer)
- Chronic lymphocytic leukemia
- Follicular lymphoma
Correct answer: Classical Hodgkin lymphoma
Reed-Sternberg cells (CD15+, CD30+, usually CD20−) are the malignant cells of classical Hodgkin lymphoma and are required for histologic diagnosis.
Question 2: The Lugano classification (updated Ann Arbor staging) for lymphoma designates Stage IV disease as:
- Involvement of two or more lymph node regions on the same side of the diaphragm
- Involvement of lymph node regions on both sides of the diaphragm
- Involvement of one extralymphatic organ without lymph node disease
- Diffuse or disseminated involvement of one or more extralymphatic organs with or without lymph node involvement (Correct answer)
Correct answer: Diffuse or disseminated involvement of one or more extralymphatic organs with or without lymph node involvement
Stage IV in the Lugano classification indicates diffuse or disseminated extranodal organ involvement (e.g., bone marrow, liver, lung parenchyma), which signals widely disseminated disease.
Question 3: R-CHOP is the standard first-line regimen for diffuse large B-cell lymphoma (DLBCL). What does the 'R' represent and what is its mechanism?
- Romidepsin — a histone deacetylase inhibitor targeting T-cell lymphomas
- Rituximab — an anti-CD20 monoclonal antibody that depletes B cells (Correct answer)
- Revlimid (lenalidomide) — an immunomodulatory agent
- Ruxolitinib — a JAK1/2 inhibitor
Correct answer: Rituximab — an anti-CD20 monoclonal antibody that depletes B cells
Rituximab is an anti-CD20 chimeric monoclonal antibody that targets CD20-expressing B cells through antibody-dependent cellular cytotoxicity and direct apoptosis, improving outcomes in DLBCL when added to CHOP chemotherapy.
Question 4: The International Prognostic Index (IPI) for aggressive non-Hodgkin lymphoma includes which of the following five adverse factors?
- Age >60, elevated LDH, ECOG PS ≥2, Stage III–IV, >1 extranodal site (Correct answer)
- Age >60, bone marrow involvement, elevated LDH, B symptoms, Stage III–IV
- Elevated LDH, low albumin, elevated beta-2 microglobulin, ECOG PS ≥2, Stage IV
- Age >60, elevated LDH, low hemoglobin, Stage III–IV, >1 nodal site
Correct answer: Age >60, elevated LDH, ECOG PS ≥2, Stage III–IV, >1 extranodal site
The five IPI factors are age >60 years, elevated serum LDH, ECOG performance status ≥2, Ann Arbor Stage III or IV, and >1 extranodal site of disease involvement.
Question 5: A patient with follicular lymphoma grade 1–2 requires CNS prophylaxis. Is this standard practice?
- Yes, all lymphoma patients require CNS prophylaxis
- No, CNS prophylaxis is not routinely indicated for low-grade follicular lymphoma (Correct answer)
- Yes, because follicular lymphoma frequently transforms to involve the CNS
- No, CNS prophylaxis is only indicated for Hodgkin lymphoma
Correct answer: No, CNS prophylaxis is not routinely indicated for low-grade follicular lymphoma
CNS prophylaxis is generally reserved for high-risk aggressive lymphomas (e.g., high-IPI DLBCL, primary testicular or renal lymphoma); indolent follicular lymphoma has very low CNS risk and does not routinely require prophylaxis.
Question 6: Burkitt lymphoma is characterized by translocation of the MYC oncogene, most commonly t(8;14). Which virus is most strongly associated with the endemic (African) form of Burkitt lymphoma?
- Human herpesvirus 8 (HHV-8)
- Human T-lymphotropic virus type 1 (HTLV-1)
- Epstein-Barr virus (EBV) (Correct answer)
- Hepatitis C virus (HCV)
Correct answer: Epstein-Barr virus (EBV)
EBV is detected in virtually all cases of endemic Burkitt lymphoma in equatorial Africa, where it acts as a cofactor alongside malaria-driven B-cell proliferation and MYC translocation.
Question 7: A patient with Hodgkin lymphoma achieves complete remission after ABVD chemotherapy. What is the standard surveillance approach regarding PET-CT imaging after treatment completion?
- PET-CT every 3 months for 5 years regardless of symptoms
- Routine PET-CT surveillance is not recommended after complete remission due to low yield and radiation risk; symptom-directed evaluation is preferred (Correct answer)
- Annual PET-CT for 10 years is standard of care
- PET-CT only if the patient develops a new palpable lymph node
Correct answer: Routine PET-CT surveillance is not recommended after complete remission due to low yield and radiation risk; symptom-directed evaluation is preferred
Current guidelines (NCCN, ESMO) do not recommend routine surveillance PET-CT after complete remission in Hodgkin lymphoma because it does not improve survival and leads to false positives and unnecessary interventions.
AOCNP Hematologic Malignancies 2
Reed-Sternberg cells, large binucleated cells with prominent 'owl-eye' nucleoli, are pathognomonic of which disease?