Practice question answers I lymphoid neoplasms

Practice questions answers

Lymphoid neoplasms I

© Jun Wang, MD, PhD

07/26/2018

1. C. Typical clinical presentation of Burkitt lymphoma. Pay attention to epidemiologic features. Genetic alteration for Burkitt lymphoma involves c-myc. Bcl2 is seen in follicular lymphoma; BRAF in hairy cell leukemia; cyclin D1 in mantle cell lymphoma; EWS in Ewing sarcoma, a tumor seen in children, characterized by small blue cells that are positive for CD99, but negative for CD45 and other lymphocytic markers such as CD10, CD20.

2. A. Burkitt lymphomais associated with EB virus. HHV 8 is associated with primary effuse lymphoma. HIV can be a factor of promoting Burkitt lymphoma, but in the group of immunodeficiency associated Burkitt lymphoma. This case is a typical endemic type Burkitt lymphoma. HPV is usually associated with carcinoma, especially squamous cell carcinoma.

3. A. Although diffuse large B cell lymphoma may have similar immunohistochemistry profile, the cells are large and more pleomorphic, in contrast with Burkitt lymphoma, which usually has monotonous intermediate sized cells with round nuclei. Ewing sarcoma is positive for CD99 but negative lymphocytic markers such as CD10, CD20, and CD45. Follicular lymphoma are positive for CD10, but almost never has a proliferative index of 100%. Adenocarcinoma is usually positive for cytokeratin.

4. B. Burkitt lymphoma usually have a round intermediate sized nuclei and proliferative index of 100%. Follicular lymphoma usually has a nodular pattern, not diffuse infiltrates. Melanomas are positive for S100. Seminomas are positive for placental alkaline phosphatase. In addition,  diffuse large B cell lymphoma is the most common testicular tumor in older man.

5. D. Light chain restriction strongly support that this is a neoplastic process. Positive CD20 reactivity is consistent with a B cell lymphoma. With the immunohistochemistry profiles of negative CD5 and CD10, this is most likely a marginal zone lymphoma, MALT type. In stomach, marginal zone lymphomas are most likely associated with H. pylori infection. Burkitt lymphomas and follicular lymphomas are positive for CD10. Diffuse large B cell lymphoma have large pleomorphic tumor cells. Chronic lymphocytic leukemia/Small lymphocytic lymphomas are positive for CD5.

6. B. See answers to questions 2 and 5.

7. C. Increased number of small lymphocytes with nuclei of soccer ball appearance is highly suggestive of chronic lymphocytic leukemia/small lymphocytic lymphoma. The immunohistochemistry profile of positive CD5, CD23, CD20 is consistent with B cell chronic lymphocytic leukemia. Acute lymphoblastic leukemia usually have an abrupt onset. The tumor cells are large and positive for TdT. Hairy cell leukemia usually presents with massive splenomegaly and tumor cells with thread-like extensions and is positive for CD11c. Mantle cell lymphoma is positive for Cyclin D1.

8. E. ZAP 70 is a commonly used marker for prognosis in patients with Chronic lymphocytic leukemia/Small lymphocytic lymphoma. Positive reactivity of ZAP 70 is indicative of a poor prognosis. Bcl2 is positive for follicular lymphoma. BRAF is mutated in hairy cell leukemia. C-myc mutation is seen in Burkitt lymphoma. Notch1 mutation is seen in T-cell acute lymphoblastic leukemia/lymphoma.

9. E. The tumor in stomach has all features of diffuse large B cell lymphoma, morphology and immunohistochemistry profiles. It is a transformation of small lymphocytic lymphoma into diffuse large B cell lymphoma, consistent with the definition of Richter syndrome. Hairy cell leukemia, mantle cell lymphoma and lymphoblastic lymphoma have different markers, see answer of question 7.

10. A. Neoplastic growth expressing CD20 growth is consistent with B cell lymphoma. With the nodular pattern, small to intermediate sized tumor cells, positive reactivity to CD10 and bcl2 confirm the diagnosis of follicular lymphoma. Hodgkin lymphoma nodular sclerosis type has bands of fibrosis that is missing in this case. In addition, binucleated Reed-Sternberg cells with prominent nucleoli that expressing CD15 and CD30 are needed for the diagnosis of any type of classical Hodgkin lymphoma. Metastatic carcinomas are usually positive for cytokeratin but negative for lymphocytic markers. Mantle cell lymphoma and SLL have different immunohistochemistry profiles, see answer to question 7.

11. E. Two commonly seen genetic abnormality involved in follicular lymphoma include bcl2 and MLL2. ALK is seen in anaplastic large cell lymphoma. BRAF is seen hairy cell leukemia. C-myc is seen in Burkitt lymphoma. CDH1 mutation is seen in lobular carcinoma of breast and signet ring carcinoma of stomach.

12. C. History of HIV, effusion with markedly atypical cells expression CD20 and absence of recognizable mass are consistent with primary effuse lymphoma, an aggressive subtype of diffuse large B cell lymphoma seen in HIV positive patients. Hodgkin lymphoma is positive for CD30. Reactive pleural effusions do not have malignant cells. Inflammatory cells seen in empyema are primarily neutrophils.

13. A. Three viruses associated with primary effuse lymphoma are HIV, EBV and HHV8. HPV is usually associated with carcinoma, especially squamous cell carcinoma. HTLV-1 is associated with adult T cell leukemia/lymphoma. Staphylococcus and streptococcus can be seen in empyema subsequent to staphylococcus or streptococcus caused pneumonia.

14. B. Although gastric MALT type marginal zone lymphomas are often associated with Helicober pylori infection, other type of gastric lymphomas do occur in patients with history of H. pylori infection. Marginal zone lymphomas are negative for both CD5 and CD10, and positive reactivity to cyclin D1 and CD5 is most consistent with mantle cell lymphoma. Follicular lymphoma is positive for CD10 and bcl2 but negative for CD5. Adenocarcinoma is positive for cytokeratin and negative for lymphocytic markers. Reactive inflammatory changes seen in peptic ulcer may have lymphocytic infiltration, but the lymphocytic population is polyclonal with a mixed kappa and lambda expression, in a background of mixed inflammatory infiltration including neutrophils.

15. C. Cyclin D1 abnormality is seen in mantle cell lymphoma. Abnormalities in bcl2 is seen in follicular lymphoma, C-myc in Burkitt lymphoma, MALT1 in marginal zone lymphoma and MIR15A/MIR16A in Chronic lymphocytic leukemia/Small lymphocytic lymphoma.

16. D. Anemia, thrombocytopenia, and neutropenia are commonly seen in hairy cell leukemia. When there is failure of bone marrow aspiration (dry tap), consider hairy cell leukemia and myelofibrosis. Both conditions may have massive splenomegaly. However, teardrop-shaped red cells are commonly seen in myelofibrosis. In addition, the presence of lymphocytes with threadlike projection is most consistent with hairy cell leukemia, in addition to positive CD11c reactivity. Histologically, tumor cells of hairy cell leukemia have a centrally located round nucleus with pale cytoplasm, a feature called “fried egg appearance”. T cell lymphoma/leukemis are positive for T cell markers such as CD3 and CD4. Acute lymphoblastic leukemia/lymphoma are positive for TdT. Chronic lymphocytic leukemia/Small lymphocytic lymphoma is characterized by small lymphocyte with soccer ball appearing nuclei, and is positive for CD5 and CD23.

17. B. ALK abnormality is seen in anaplastic large cell lymphoma; Cyclin D1 in mantle cell lymphoma; JAK2 in myeloproliferative neoplasms including polycythemia vera, essential thrombocythemia and primary myelofibrosis.

18. B. Abrupt onset is compatible with acute leukemia. The presence of large amount large agranular white cells is consistent with acute lymphoblastic leukemia/lymphoma. The positive reactivity to TdT and CD3 confirms the presence of T cell lymphoblast. B-cell acute lymphoblastic leukemia/lymphoma is positive for CD79a, a B cell marker. Chronic lymphocytic leukemia/Small lymphocytic lymphoma usually has an indolent clinical course and is characterized by large amount of small lymphocytes. Hairy cell leukemia is characterized by massive splenomegaly and tumor cells with more cytoplasm, threadlike projections, and negative reactivity to CD3 and TdT. Hodgkin lymphoma may present with a mediastinum mass as seen in this patient. However, Reed-Stenberg cells and proper immunohistochemistry profiles are needed for the diagnosis.

19. D. BRAF abnormality is seen in hairy cell leukemia; c-myc in Burkitt lymphoma; MYD88 in lymphoplasmacytic lymphoma; RUNX1 in B-cell acute lymphoblastic leukemia/lymphoma and AML with t(8;21)(q22;q22.1).

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