Practice question answers II lymphoid

Practice questions answers

Lymphoid neoplasms II

© Jun Wang, MD, PhD

07/26/2018

1. D. Young adults present with cervical lymphadenopathy is highly suspicious for Hodgkin lymphoma. The microscopic features and immunohistochemistry profiles are typical for a classical Hodgkin lymphoma, nodular sclerosis type. Anaplastic large-cell lymphoma has markedly atypical tumor cells with horseshoe or donut-shape large cells that are positive for ALK and CD30. Diffuse large B cell lymphoma is positive for CD20. Follicular lymphoma is positive for CD10 and CD20, but negative for CD15 and CD30. Both follicular lymphoma and reactive lymphadenopathy do not have Reed-Sternber cells.

2. A. Hodgkin lymphoma is associated with EBV. HHV8 is associated with primary effuse lymphoma and Kaposi sarcoma. HIV post a risk factor for various lymphomas, but most likely would not be directly associated with Hodgkin lymphoma. HPV is associated with carcinomas, especially squamous cell carcinoma. HTLV-1 is associated with adult T-cell leukemia/lymphoma.

3. A. The morphology of tumor cell, multilobated nuclei (flower cells), and positive reactivity to T cell markers CD3 and CD4, are most consistent with adult T-cell leukemia/lymphoma. Anaplastic large-cell lymphoma may have leukemic phase, but it is usually positive for ALK. Chronic lymphocytic leukemia/Small lymphocytic lymphoma is a B cell leukemia that is positive for CD5, CD20 but negative for T cell markers. Diagnosis of multiple myeloma needs evidence of monoclonal gammaglobulinopathy, bone damage and tumoral growth of plasma cells that are positive for CD138. Sezary syndrome has similar immunohistochemistry profiles (positive for CD3 and CD4) for their cerebriform neoplastic lymphocytes, but it usually has skin lesions.

4. D. See answer to question 2.

5. B. Anaplastic large-cell lymphoma is characterized by markedly atypical tumor cells with horseshoe and donut-shaped nuclei, which are positive for ALK and CD30.  Adult T-cell leukemia/lymphomas are negative for ALK. Diffuse large B cell lymphomas are positive for CD20. Classical Hodgkin lymphomas are negative for ALK and CD45. Carcinomas are positive for cytokeratin.

6. A. Abnormality of ALK is seen in anaplastic large-cell lymphoma; BRAF in hairy cell leukemia; Cyclin D1 in mantle cell lymphoma; MYD 88 in lymphoplasmacytic lymphoma; p16 in squamous cell carcinoma, especially HPV associated.

7. E. Sezary syndrome is characterized by a triad of diffuse erythroderma, generalized lymphadenopathy and the presence of clonal neoplastic T cells with cerebriform nuclei (Sezary cells) in skin, lymph nodes or blood. These tumor cells are CD4 positive T cells, and negative for CD8 and B cell marker CD20. Adult T-cell leukemia/lymphoma is characterized by flower like tumor cells that is also positive for CD3 and CD4, but negative for CD20. Anaplastic large-cell lymphomas are positive for ALK. Chronic lymphocytic leukemia/Small lymphocytic lymphoma is characterized by small lymphocytes that are positive for CD5, CD20 and CD23, but negative for T cell markers CD3 and CD4. Reactivity lymphadenopathy usually has a mixed lymphocytic population without cerebriform lymphocytes.

8. D. CD4 positive monoclonal T cell neoplasms with cerebriform nuclei limited to skin is most consistent with mycosis fungoides. Anaplastic large-cell lymphoma is characterized by large malignant lymphocytes with marked pleomorphism, including horseshoe and donut-shaped nuclei. Dermatophytosis may have various presentation but is positive for fungal studies and have a polyclonal lymphocytic and neutrophilic infiltrate instead of monoclonal lymphocytic proliferation. Marginal zone lymphoma is a B cell lymphoma that is positive for CD20, but negative for CD3 and CD4. Sezary syndrome has widespread erythroderma, diffuse lymphadenopathy in addition to the presence of Sezary cells.

9. D. Plasma cells are characterized by eccentrically located round nuclei with dense chromatin forming a “clock-face” appearing, and a perinuclear pallor area. They are positive for CD38, CD138, and negative for CD3, CD20. Cells with grapelike cytoplasmic inclusions (Mott cells) can be seen in plasma cell neoplasms. The presence of plasma cells for more than 10% in a bone marrow biopsy as well as evidence of bone destructions, renal failure are consistent with multiple myeloma. Adult T-cell leukemia/lymphoma is characterized by flower cells and positive reactivity to CD3, but negative for CD20, CD38 and CD138. Diffuse large B cell lymphomas are positive for CD30, but negative for CD138. Chronic lymphocytic leukemia/Small lymphocytic lymphoma is positive for CD5, CD20 and CD23, but negative for T cell markers such as CD3. Carcinomas are positive for cytokeratin.

10. B. Most commonly seen M protein in patients with multiple myeloma is IgG. Elevated CEA is commonly associated with colon adenocarcinoma etc, PSA in prostate adenocarcinoma. The elevated serum calcium is most likely caused by tumor associated bone destruction, not hyperparathyroidism. Indeed, his parathyroid hormone is probably lower than normal (secondary hypoparathyroidism).

11. A. Renal insufficiency in patients with plasma cell neoplasms is most likely associated with toxicity of light chains (Bence Jones proteins) to renal tubules.

12. D. The presence of amorphous deposit that is positive for Congo Red is consistent with amyloid. Immunoflourescence studies confirms these are kappa light chains, that is most likely resulted from a plasma cell growth instead of reactive process to chronic inflammation. Diabetic glomerular injury is characterized by diffuse capillary basement membrane thickening, diffuse and nodular glomerulosclerosis, etc. Hypertensive nephropathy is characterized by hyaline arteriosclerosis, thickening of capillary basement membrane,, glomerular sclerosis. Neither of these two has amyloid deposit. 

13. C. Presence of amyloid deposit in kidney raise the concern of plasma dyscresia, that need to be worked up by immunofixation for monoclonal gammopathy. Microalbumin, glucose tolerance tests are commonly used if diabetes is in concern. Immune complex deposit in kidney is usually associated with membranous nephropathy, lupus nephritis, and postinfectious glomerulonephritis. These three usually do not have amyloid deposit.

14. D. This patient has symptoms of hyperviscosity syndrome due to elevated levels of IgM. Biopsy of his cervical lymph nodules reveal lymphoplasmacytic lymphoma characterized by lymphoma with plasmacytic differentiation. The tumor cells are positive for both B cell markers CD19, CD20 and plasma cell marker CD138. Light chain restriction is consistent with monoclonal proliferation. Anaplastic large-cell lymphoma have large bizarre nuclei and are positive for ALK. Diffuse large B cell lymphoma usually has large pleomorphic cells are positive for CD20, but negative for CD138. Hodgkin lymphoma has Reed-Sternberg cells that are usually CD30 positive. Multiple myelomas usually are negative for CD19 and CD20.

15. C. Hyperviscosity associated with retinopathy is usually resulted from distended and tortuous retinal veins with hemorrhage and exudates.

16. D. Abnormality of ALK is seen in anaplastic large-cell lymphoma; bcl2 in follicular lymphoma; cyclin D1 in mantle cell lymphoma, STAT3 may be seen in large granular lymphocytic leukemia.

17. C. The patient has monoclonal gammopathy, but no evidence of bone lesion. The total serum IgM is elevated but not to the level of multiple myeloma (3g/dL). The clinical presentation is consistent with paraproteinemic neuropathy, a presentation most commonly seen in IgM gammopathy. These findings are consistent with monoclonal gammopathy of undetermined significance. Lymphoplasmacytic lymphoma and plasmacytoma have discrete mass. Multiple myeloma has higher gammaglobulin levels, bone and other organ damage, as well as at a level of 10% or more of marrow cellularity, if discrete mass is not present.

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