Practice questions answers Lymphoid neoplasms IV

Practice questions answers

Lymphoid neoplasms IV

 

© Jun Wang, MD, PhD

 

1. D. This case is characterized by a rapid growing lymphoid lesion composed of small to medium sized round lymphocytes. Large cells with pale cytoplasm are scattered among these lymphocytes, forming a starry sky appearance. The expressions of CD10, CD19 and CD20, but not ALK, CD3, CD5, CD23, CD30, CD56 and cytokeratin are consistent with a B-cell lymphoma. Proliferative index by Ki67 100% is characteristic for Burkitt lymphoma, commonly with c-myc mutation. ALK is abnormal in anaplastic large cell lymphoma. BRAF mutation can be seen in hairy cell leukemia, melanocytic nevus and melanoma. IGH-CCND1 is a result of t(11;14)(q13;q32) in mantle cell lymphoma. MYD88 mutation can be seen in lymphoplasmacytic lymphoma.

2. A. EBV is associated with various lymphomas, including classical Hodgkin lymphoma, extranodal T/NK cell lymphoma, Burkitt lymphoma and primary effusion lymphoma. H. pylori can be associated with marginal zone lymphoma and gastric adenocarcinoma. HHV8 is associated with primary effusion lymphoma and Kaposi sarcoma. HTLV1 is associated with adult T cell leukemia/lymphoma. HPV may be associated with various carcinomas, including oral cavity squamous cell carcinoma, anal cannel squamous cell carcinoma, cervical cancers, and certain cutaneous squamous cell carcinoma, but not leukemia/lymphoma.

3. B. See discussion of question 1. Anaplastic large cell lymphoma is characterized by markedly atypical and bizarre large cells expressing T cell markers, may be positive for ALK, but usually negative for B cell markers. Diffuse large B-cell lymphoma tends to have sheets of large atypical B cells, without follicular pattern. Hodgkin lymphoma has RS cells or popcorn cells. Mantle cell lymphoma is positive for CD5 and cyclin D1, but negative for CD10 and bcl2. Marginal zone lymphoma is positive for CD20, but negative for CD5, CD10 and cyclin D1. Small lymphocytic lymphoma is in the same category of chronic lymphocytic leukemia, characterized by proliferation of small lymphocytes with “soccer ball” appearing nuclei, and expression CD5 and CD23, but not cyclin D1. None of these has a proliferation index of 100%.

4. B. This case is characterized by anemia, hypercalcemia and multiple lytic bone lesions. Biopsy of the bone lesions reveals sheets of cells with eccentric nuclei containing clumped chromatin, forming a “clock face” appearance. These cells are positive for plasma cell markers CD38, CD138, and produce kappa light chain. They are negative for T cell markers CD3, CD4 and B cell markers CD19, CD20, and epithelial marker cytokeratin. These features are consistent with plasmacytomas, since these cells are more than 10% in the biopsy. The presence of plasmacytomas, in a patient with evidence of severe bone damage, kidney failure, anemia, or hypercalcemia, is diagnostic of multiple myeloma. M protein is monoclonal immunoglobulin. Bence Jones proteins are free light chains without attached heavy chains. Both are commonly seen in multiple myeloma. Abnormal serum folate levels are commonly seen in megaloblastic anemia. Abnormal TIBC is seen in anemia associated with chronic disease or iron deficiency anemia. Elevated parathyroid hormone due to primary hyperparathyroidism may cause lytic bone lesion. However, these lesions have increased multinucleated osteoclasts, not plasma cells. PSA is elevated in prostate adenocarcinoma that may have bone metastasis. However, metastatic carcinoma to any location would be positive for cytokeratin. 

5. E. Lytic bone lesions caused by multiple myeloma are commonly associated with elevated RANKL, resulting in osteoclast activation. Amyloid deposit is characterized by amorphous deposit that is positive for Congo Red. Metastatic adenocarcinoma is positive for cytokeratin. Myelofibrosis is characterized by hypocellular bone marrow with fibrosis. Activation of osteoblasts result in bone formation, not destruction.

6. D. Multiple myeloma and other disorders that lead to elevated serum protein may cause Rouleaux of red cells, due to neutralization of RBC surface electric charges. Faggot cells are characterized by abundant Auer rods in neoplastic promyelocytes, a feature of APL with t(15;17)(q22;q12) involving RARA. Giant platelet and nucleated red cells are commonly seen with bone marrow damages. Target cells can be seen in hemoglobin abnormalities, including thalassemia, and HbE disease.

7. E. See discussion of questions 3 and 4. Monoclonal gammopathy of undetermined significance has less than 10% clonal plasma cells in biopsy. Osteosarcoma is characterized by malignant tumor with bone formation directly by tumor cells.

8. E. See discussion of question 4.

9. A. This case is characterized by markedly atypical cells that are positive for CD3, CD30 and CD45, but not cytokeratin and B-cell markers CD19 and CD20. Bizarre nuclei with positive CD30 reactivity is most compatible with anaplastic large cell lymphoma, a tumor commonly positive for ALK. Bcl2 might be positive for a few B cell lymphomas, including diffuse large B-cell lymphoma and follicular lymphoma. CD15 might be positive for RS cells of classical Hodgkin lymphoma. EBV is associated with various lymphomas, including classical Hodgkin lymphoma, extranodal T/NK cell lymphoma, Burkitt lymphoma and primary effusion lymphoma. HHV8 is positive for primary effusion lymphoma and Kaposi sarcoma. However, these markers tend to be negative for anaplastic large cell lymphoma.

10. A. ALK translocation is commonly seen in ALK positive anaplastic large cell lymphoma. BRAF mutation can be seen in hairy cell leukemia, melanocytic nevus and melanoma. IGH-CCND1 is a result of t(11;14)(q13;q32) in mantle cell lymphoma. MYD88 mutation can be seen in lymphoplasmacytic lymphoma. JAK2 mutation is seen in polycythemia vera and essential thrombocythemia. NF-kB mutation can be seen in classical Hodgkin lymphoma. Notch1 mutation is commonly seen in T-cell acute lymphoblastic leukemia.

11. B. See discussion of question 9. Adult T cell leukemia/lymphoma is negative for CD30 and ALK. Diffuse large B-cell lymphoma tends to have sheets of large atypical B cells, without follicular pattern and are positive for B cell markers. Extranodal T/NK cell lymphoma is positive for EBV and one of the NK cell markers, such as CD56 and CD57. Classical Hodgkin lymphoma has RS cells in a background of inflammatory cell infiltrates. These RS cells are positive for CD15 and CD30, but negative for CD45, T cell markers and ALK. Nodular lymphocyte predominant Hodgkin lymphoma has popcorn cells in a background of lymphocytes. These popcorn cells are positive for CD45, CD20, but negative for ALK, CD15 and CD30.

12. A. This case is characterized by a rapid growing, aggressive tumor composed of markedly atypical tumor cells positive for T cell markers CD2, CD3, CD5, NK cell marker CD56 and EBV, but negative for CD10, CD30, B-cell markers CD19 and CD20, and plasma cell marker CD38. These findings are consistent with extranodal T/NK cell lymphoma, that is associated with EBV infection. Also see discussion of question 2.

13. D. See discussion of questions 11 and 12.

14. F. This case is characterized by indolent clinical process with anemia, neutropenia and presence of large atypical lymphocytes with cytoplasmic granules. These cells are positive for both T cell markers CD2, CD3, CD5 and NK cell marker CD56, but negative for CD10, B cell marker CD20, and blast markers CD33, CD34, and TdT. These findings, in addition to the history of rheumatoid arthritis, are consistent with T cell large granular lymphocytic leukemia, a disorder that STAT3 mutation is commonly seen. See discussion of questions 1 and 10.

15. F. See discussion of question 14. Blast markers CD33, CD34 and/TdT are positive in acute myeloid leukemia, or acute lymphoblastic leukemia. Adult T cell leukemia/lymphoma has florid cells and tend to be negative for CD56. Chronic lymphocytic leukemia is characterized by increased number of small lymphocytes that are positive for CD20, CD5 and CD23. Hairy cell leukemia is a B-cell neoplasm characterized by pancytopenia, and the presence lymphocytes with hairy projects.

16. D. This case is characterized by large tumor cells with binucleated cells with irregular nuclear membrane and prominent red nucleoli, in a background of inflammatory cells. This is most likely RS cells of classical Hodgkin lymphoma, positive for CD15, CD30, and negative for CD3, CD45. Small lymphocytic neoplasm positive for CD5, CD20, CD23, negative for CD10, CD30 and Cyclin D1 is consistent with chronic lymphocytic leukemia/small lymphocytic lymphoma. Positive for CD5, CD20, and cyclin D1, negative for CD10, CD23 can be seen for mantle cell lymphoma. Immunohistochemistry positive for CD10, CD20, CD45, negative for CD5, CD23, CD30 can be seen for follicular lymphoma if nodular pattern, or diffuse large B-cell lymphoma if sheets of large atypical cells. Immunohistochemistry profile oositive for CD20, CD45, negative for CD5, CD10 and CD23 can be seen in marginal zone lymphoma. Immunohistochemistry profile positive for CD30, CD45, ALK, and negative for CD10, CD20 can be seen in anaplastic large cell lymphoma.

17. A. See discussion of question 2.

18. D. NF-kB mutation can be seen in classical Hodgkin lymphoma. See discussion of questions 10 and 14.

19. C. See discussion of questions 3 and 16. Acute lymphadenitis does not have RS cells.

20. F. This case is characterized by popcorn appearing cells in a background of almost monotonous lymphocytes. This pattern is consistent with nodular lymphocyte predominant Hodgkin lymphoma. These popcorn cells are positive for CD20 and CD45, but negative for CD15 and CD30. See discussion of question 16.

21. F. See discussion of questions 3, 16, 19 and 20.

 

 

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