Practice questions I lymphoid neoplasms

Practice questions
Lymphoid neoplasms I

© Jun Wang, MD, PhD

07/26/2018

1. Use this case for the next three questions. A 5-year-old boy from southeastern Asia presents with rapidly enlarging mass on his left lower face. Radiological examination reveals a tumor invading into left mandible with bone destruction. Biopsy reveals sheets of monotonous intermediated sized tumor cells with round nuclei and scant cytoplasm. Among these tumor cells are many larger cells with abundant cytoplasm containing darkly stained cell debris. Immunohistochemistry studies reveal these tumor cells are positive for CD10, CD20, but negative for CD3, CD5, CD99 and pan cytokeratin. Proliferative index by Ki67 is approximately 100%. What gene is most likely altered in these tumor cells?
A.                Bcl2
B.                 BRAF
C.                 C-myc
D.                Cyclin D1
E.                 EWS

2. A 5-year-old boy from southeastern Asia presents with rapidly enlarging mass on his left lower face. Radiological examination reveals a tumor invading into left mandible with bone destruction. Biopsy reveals sheets of monotonous intermediated sized tumor cells with round nuclei and scant cytoplasm. Among these tumor cells are many larger cells with abundant cytoplasm containing darkly stained cell debris. Immunohistochemistry studies reveal these tumor cells are positive for CD10, CD20, but negative for CD3, CD5, CD99 and pan cytokeratin. Proliferative index by Ki67 is approximately 100%. What viral infection is most likely associated with this abnormality?
A.                Epstein Barr virus
B.                 Human herpes virus 8
C.                 Human immunodeficiency virus
D.                Human papilloma virus

3. A 5-year-old boy from southeastern Asia presents with rapidly enlarging mass on his left lower face. Radiological examination reveals a tumor invading into left mandible with bone destruction. Biopsy reveals sheets of monotonous intermediated sized tumor cells with round nuclei and scant cytoplasm. Among these tumor cells are many larger cells with abundant cytoplasm containing darkly stained cell debris. Immunohistochemistry studies reveal these tumor cells are positive for CD10, CD20, but negative for CD3, CD5, CD99 and pan cytokeratin. Proliferative index by Ki67 is approximately 100%. What is the most likely diagnosis?
A.                Burkitt lymphoma
B.                 Diffuse large B cell lymphoma
C.                 Ewing sarcoma
D.                Follicular lymphoma
E.                 Poorly differentiated adenocarcinoma

4. A 70-year-old man presents with swelling of his right testicle for 3 months. He has fatigue, night sweat for one month, and lost 20 pound unintentionally. His past medical history include melanoma of right forearm 5 year ago, that was resected. Laboratory tests are within normal range. A biopsy was performed and reveals testicular parenchyma diffusely infiltrated by atypical cells with large irregular nuclei. Nucleoli can be seen in some cells. Immunohistochemistry studies reveal these cells are positive for bcl2, CD20 and CD45, negative for cytokeratin, S100, alpha fetoprotein, hCG and placental alkaline phosphatase. Proliferative index per ki67 is approximately 90%. What is most likely the diagnosis?
A. Burkitt lymphoma
B. Diffuse large B cell lymphoma
C. Follicular lymphoma
D. Metastatic melanoma
E. Seminoma

5. Use this case for the next two questions. A 55-year-old man presents with worsening central epigastric discomfort for a month. He has a history of antrum peptic ulcer for 3 years. Physical examination and laboratory tests are unremarkable. Gastroscopic examination reveals a 5 cm ulcer at the lesser curvature side of antrum. Microscopic examination of the ulcer reveal diffuse lymphocytic and plasmacytic infiltration. Scattered neutrophils are seen. The lymphocytes form vague nodular appearance and have intermediate sized round nuclei. No nucleoli are seen. Some lymphocytes are seen infiltrating pyloric glands. Immunohistochemistry studies reveal that these lymphocytes express CD20, and predominantly kappa light chain. They are negative for CD3, CD5 and CD10. What is the most likely diagnosis?
A.    Burkitt lymphoma
B.     Diffuse large B cell lymphoma
C.     Follicular lymphoma
D.    Marginal zone lymphoma
E.     Small lymphocytic lymphoma

6. A 55-year-old man presents with worsening central epigastric discomfort for a month. He has a history of antrum peptic ulcer for 3 years. Physical examination and laboratory tests are unremarkable. Gastroscopic examination reveals a 5 cm ulcer at the lesser curvature side of antrum. Microscopic examination of the ulcer reveal diffuse lymphocytic and plasmacytic infiltration. Scattered neutrophils are seen. The lymphocytes form vague nodular appearance and have intermediate sized round nuclei. No nucleoli are seen. Some lymphocytes are seen infiltrating pyloric glands. Immunohistochemistry studies reveal that these lymphocytes express CD20, and predominantly kappa light chain. They are negative for CD3, CD5 and CD10.
A. Epstein Barr virus
B. Helicobacter pylori
C. Human herpes virus 8
D. Human immunodificeincy virus
E. Human papilloma virus

7. Use this case for the next three questions. A 65-year-old woman presents with fatigue for three months. Her past history includes hypertension, type 2 diabetes, and coronary artery disease. Her physical examination are unremarkable except a blood pressure of 180/100 mmHg. Her CBC reveals a hemoglobin of 7.5 g/dl, white count 21 × 109/L, an absolute lymphocyte count of 20 × 109/L. Peripheral blood smear reveal majority of the lymphocytes are small lymphocytes with scant cytoplasm, round nuclei and clumped chromatin. Many of these nuclei have a soccer ball appearance. Flow cytometry studies reveal these lymphocytes are positive for CD5, CD20, CD23 with lambda chain restriction, and negative for TdT, CD10, CD11c and cyclin D1. Myeloid series and platelets are unremarkable. A complete work up reveals a 2 cm mass at her stomach. A biopsy reveals gastric mucosa infiltrated with atypical lymphocytes with similar morphology and immunohistochemistry profiles of those found in her peripheral blood. What is the most likely diagnosis?
A. Acute B cell lymphoblastic leukemia
B. Acute T cell lymphoblastic leukemia
C. Chronic lymphocytic leukemia
D. Hairy cell leukemia
E. Mantle cell lymphoma, leukemic phase

8. A 65-year-old woman presents with fatigue for three months. Her past history also include hypertension, type 2 diabetes, and coronary artery disease. Her physical examination are unremarkable except a blood pressure of 180/100 mmHg. Her CBC reveals a hemoglobin of 7.5 g/dl, white count 21 × 109/L, an absolute lymphocyte count of 20 × 109/L. Peripheral blood smear reveal majority of the lymphocytes are small lymphocytes with scant cytoplasm, round nuclei and clumped chromatin. Many of these nuclei have a soccer ball appearance. Flow cytometry studies reveal these lymphocytes are positive for TdT, CD5, CD20, CD23 with lambda chain restriction, and negative for CD10, CD11c and cyclin D1. Myeloid series and platelets are unremarkable. A complete work up reveals a 2 cm mass at her stomach. A biopsy reveals gastric mucosa infiltrated with atypical lymphocytes with similar morphology and immunohistochemistry profiles of those found in her peripheral blood. What is a useful marker to assess her prognosis?
A. Bcl2
B. BRAF
C. C-myc
D. Notch1
E. Zap 70

9. A 65-year-old woman presents with fatigue for three months. She has a history of right breast invasive ductal carcinoma that was treated with lumpectomy and chemotherapy 5 years ago. Her past history also include hypertension, type 2 diabetes, and coronary artery disease. Her physical examination are unremarkable except a blood pressure of 180/100 mmHg. Her CBC reveals a hemoglobin of 7.5 g/dl, white count 21 × 109/L, an absolute lymphocyte count of 20 × 109/L. Peripheral blood smear reveal majority of the lymphocytes are small lymphocytes with scant cytoplasm, round nuclei and clumped chromatin. Many of these nuclei have a soccer ball appearance. Flow cytometry studies reveal these lymphocytes are positive for CD5, CD20, CD23 with lambda chain restriction, and negative for TdT, CD10, CD11c and cyclin D1. Myeloid series and platelets are unremarkable. A complete work up reveals a 2 cm mass at her stomach. A biopsy reveals gastric mucosa infiltrated with atypical lymphocytes with similar morphology and immunohistochemistry profiles of those found in her peripheral blood. During a follow up workup 6 months later, the gastric mass enlarged to 4 cm. Biopsy reveal diffuse infiltrate of atypical cells with large irregular nuclei, some of them have prominent nucleoli. Immunohistochemistry studies reveal these large cells are positive for CD20, but negative for CD3, CD11c and cyclin D1. What is the most likely diagnosis now?
A. Blastoid change of mantle cell lymphoma
B. Local infiltration of hairy cell leukemia
C. Local involvement of acute B cell lymphoblastic leukemia
D. Local involvement of acute T cell lymphoblastic leukemia
E. Richter syndrome

10. Use this case for the next two questions. A 57-year-old woman presents with a slowly growing right axillary mass for 10 months. She denies any other symptoms. She has history of invasive lobular carcinoma of her right breast 2 years ago and received lumpectomy and chemotherapy. Her past medical history is otherwise unremarkable. Physical examination reveals a 3 cm firm mass that is movable in her right axilla. Her laboratory tests including CBC are within normal range. The mass was resected and microscopically it is a lymph node with nodular growth of small to intermediate sized cells with scant cytoplasm and single slightly irregular nuclei. The center of these nodules are lighter that periphery. Between these nodules are small lymphocytes. No neutrophils or eosinophils are seen. Immunohistochemistry studies reveal the cells in these nodules are positive for CD10, CD20, bcl2, but negative for cytokeratin, CD5, CD23, CD30 and cyclin D1. What is the most likely diagnosis?
A. Follicular lymphoma
B. Hodgkin lymphoma, nodular sclerosis type
C. Mantle cell lymphoma
D. Metastatic lobular carcinoma
E. Small lymphocytic lymphoma

11. A 57-year-old woman presents with a slowly growing right axillary mass for 10 months. She denies any other symptoms. She has history of invasive lobular carcinoma of her right breast 2 years ago and received lumpectomy and chemotherapy. Her past medical history is otherwise unremarkable. Physical examination reveals a 3 cm firm mass that is movable in her right axilla. Her laboratory tests including CBC are within normal range. The mass was resected and microscopically it is a lymph node with nodular growth of small to intermediate sized cells with scant cytoplasm and single slightly irregular nuclei. The center of these nodules are lighter that periphery. Between these nodules are small lymphocytes. No neutrophils or eosinophils are seen. Immunohistochemistry studies reveal the cells in these nodules are positive for CD10, CD20, bcl2, but negative for CD5, CD23, CD30 and cyclin D1. Abnormality of what gene is most likely found in these cells?
A. ALK
B. BRAF
C. C-myc
D. CDH1
E. MLL2

12. Use this case for the next two questions. A 65-year-old man presents with fatigue, coughing with white sputum, progressive dyspnea and vague dull chest pain for a few weeks. He states that he has lost 10 pound recently. He does not have fever nor night sweating. He has been HIV positive for 30 years. His past medical history is otherwise unremarkable. Physical examination reveals inaudible breath sounds with a dull percussion of his left chest. Laboratory tests are unremarkable except a reduced CD4+ lymphocyte count. Radiological examination reveals left pleural effuse but no mass is found. Diagnostic thoracentesis was performed. Microbiological studies of his pleural effusion are negative. Microscopic examination of the pleural effusion reveals abundant markedly atypical cells with large irregular nuclei, and cytoplasmic vacuoles. A few neutrophils are seen. Immunohistochemistry studies reveal these atypical cells are positive for CD30, CD45 and CD138, but negative for CD20. In situ hybridization for EB virus is positive. What is the most likely diagnosis?
A. Empyema
B. Hodgkin lymphoma
C. Primary effusion lymphoma
D. Reactive pleural effusion due to EB virus associated pneumonia
E. Reactive pleural effusion due to pneumocystis jiroveci pneumonia

13. A 65-year-old man presents with fatigue, coughing with white sputum, progressive dyspnea and vague dull chest pain for a few weeks. He states that he has lost 10 pound recently. He does not have fever nor night sweating. He has been HIV positive for 30 years. His past medical history is otherwise unremarkable. Physical examination reveals inaudible breath sounds with a dull percussion of his left chest. Laboratory tests are unremarkable except a reduced CD4+ lymphocyte count. Radiological examination reveals left pleural effuse but no mass is found. Diagnostic thoracentesis was performed. Microbiological studies of his pleural effusion are negative. Microscopic examination of the pleural effusion reveals abundant markedly atypical cells with large irregular nuclei, and cytoplasmic vacuoles. A few neutrophils are seen. Immunohistochemistry studies reveal these atypical cells are positive for CD30, CD45 and CD138, but negative for CD20. In situ hybridization for EB virus is positive. Besides HIV and EB virus, what microorganism is associated with his condition?
A. Human herpes virus 8
B. Human papillomavirus
C. Human T cell leukemia virus type I
D. Staphylococcus aureus
E. Streptococcus pneumoniae

14. Use this case for the next two questions. A 53-year-old man presents with epigastric discomfort for a year. He has a history of Helicobacter associated gastric peptic ulcer 5 years ago, and was treated with antibiotics. His history is otherwise unremarkable. Physical examination reveals a tender mass at his left supraclavicular region measuring 1.5 cm in greatest dimension. His laboratory tests are all within normal range. Gastroscopic examination reveals a 2.5 cm ulcer at his antrum. Biopsy of the mass reveal gastric mucosa with diffuse infiltrate of small to intermediate sized cells with scant cytoplasm and irregular dark nuclei. Immunohistochemistry studies reveal these cells are positive for CD5, CD20, cyclin D1 and kappa light chain, but negative for cytokeratin, CD3, CD10 and bcl 2. Biopsy of the supraclavicular mass reveal a lymph node with various sizes of follicles without epithelioid cells. Flow cytometry studies of tissue from the supraclavicular lymph node reveal polyclonal lymphoid population. What is the most likely diagnosis of the gastric lesion?
A. Follicular lymphoma
B. Mantle cell lymphoma
C. Marginal zone lymphoma, MALT type
D. Poorly differentiated adenocarcinoma with supraclavicular lymph node metastasis
E. Recurrent peptic ulcer

15. A 53-year-old man presents with epigastric discomfort for a year. He has a history of Helicobacter associated gastric peptic ulcer 5 years ago, and was treated with antibiotics. His history is otherwise unremarkable. Physical examination reveals a tender mass at his left supraclavicular region measuring 1.5 cm in greatest dimension. His laboratory tests are all within normal range. Gastroscopic examination reveals a 2.5 cm ulcer at his antrum. Biopsy of the mass reveal gastric mucosa with diffuse infiltrate of small to intermediate sized cells with scant cytoplasm and irregular dark nuclei. Immunohistochemistry studies reveal these cells are positive for CD5, CD20, cyclin D1 and kappa light chain, but negative for cytokeratin, CD3, CD10 and bcl 2. Biopsy of the supraclavicular mass reveal a lymph node with various sizes of follicles without epithelioid cells. Flow cytometry studies of tissue from the supraclavicular lymph node reveal polyclonal lymphoid population. Abnormality of what gene is associated with his gastric lesion?
A. Bcl 2
B. C-myc
C. Cyclin D1
D. MALT1
E. MIR15A/MIR16A

16. Use this case for the next two questions. A 48-year-old man presents with fatigue, easy bruise and left upper abdomen discomfort for 3 months. His past history is unremarkable. Physical examination reveals enlarged spleen extended 6 cm below the costal margin. His CBC reveals a hemoglobin of 7.5 g/dl, platelet count of 70 x 109/L. His white cell count is within normal range, with an absolute neutrophil count at 0.7 x 109/L (normal 2-8 x 109/L). Microscopic examination of peripheral blood smears reveals slightly enlarged lymphoid cells with round to oval nuclei and thread like cytoplasmic extensions. The red cells and platelets are morphologically unremarkable. Bone marrow aspiration was not successful. Bone marrow core biopsy reveals hypercellular marrow with sheets of intermediate sized cells with centrally located round nuclei and pale cytoplasm. Immunohistochemistry studies reveal these cells are positive for CD20, CD11c, but negative for TdT, CD3, CD5, CD10, CD23 and cyclin D1. Focally there are evidence of marrow fibrosis. What is the most likely diagnosis?
A. Acute B cell lymphoblastic lymphoma
B. Adult T cell leukemia
C. Chronic lymphocytic leukemia
D. Hairy cell leukemia
E. Primary myelofibrosis

17. A 48-year-old man presents with fatigue, easy bruise and left upper abdomen discomfort for 3 months. His past history is unremarkable. Physical examination reveals enlarged spleen extended 6 cm below the costal margin. His CBC reveals a hemoglobin of 7.5 g/dl, platelet count of 70 x 109/L. His white cell count is within normal range, with an absolute neutrophil count at 0.7 x 109/L (normal 2-8 x 109/L). Microscopic examination of peripheral blood smears reveals slightly enlarged lymphoid cells with round to oval nuclei and thread like cytoplasmic extensions. Bone marrow aspiration was not successful. Bone marrow core biopsy reveals hypercellular marrow with sheets of intermediate sized cells with centrally located round nuclei and pale cytoplasm. The red cells and platelets are morphologically unremarkable. Immunohistochemistry studies reveal these cells are positive for CD20, CD11c, but negative for CD3, CD5, CD10, CD23 and cyclin D1. Focally there are evidence of marrow fibrosis. Abnormality of what gene is most likely associated with his condition?
A. ALK
B. BRAF
C. Cyclin D 1
D. JAK2
E. MPL

18. Use this case for the next two questions. A 21-year-old man presents with productive cough, nose bleeding and low-grade fever for a month. He has night sweating and has lost 10 lb. He was originally diagnosed as bronchitis and received antibiotics therapy without improvement. His history is otherwise unremarkable. Physical examination reveals tachypnea (25/min) and tachycardia (140/min). There is no palpable lymphadenopathy or organomegaly. Laboratory tests reveal hemoglobin of 8.4 g/dL (normal 13-18 g/dL), platelet of 35 x 109/L (normal 140-450 x 109/L), white count 11 x 109/L. Smear of peripheral blood reveal many large cells with scant agranular cytoplasm. Per flow cytometry studies, these large cells are positive for CD3, TdT, but negative for CD79a. Radiological studies reveal a 5 cm mass in his posterior mediastinum. Bone marrow aspiration reveals more than 30% are similar large cells that have the same flow cytometry findings. Biopsy of the mediastinum mass reveals a tumor with sheets of tumor cell with similar morphology and immunohistochemistry profiles as those seen in peripheral blood and marrow. What is most likely the diagnosis?
A. Acute B cell lymphoblastic leukemia
B. Acute T cell lymphoblastic leukemia
C. Chronic lymphocytic leukemia
D. Hairy cell leukemia
E. Hodgkin lymphoma

19. A 21-year-old man presents with productive cough, nose bleeding and low-grade fever for a month. He has night sweating and has lost 10 lb. He was originally diagnosed as bronchitis and received antibiotics therapy without improvement. His history is otherwise unremarkable. Physical examination reveals tachypnea (25/min) and tachycardia (140/min). There is no palpable lymphadenopathy or organomegaly. Laboratory tests reveal hemoglobin of 8.4 g/dL (normal 13-18 g/dL), platelet of 35 x 109/L (normal 140-450 x 109/L), white count 11 x 109/L. Smear of peripheral blood reveal many large cells with scant agranular cytoplasm. Per flow cytometry studies, these large cells are positive for CD3, TdT, but negative for CD79a. Radiological studies reveal a 5 cm mass in his posterior mediastinum. Bone marrow aspiration reveals more than 30% are similar large cells that have the same flow cytometry findings. Biopsy of the mediastinum mass reveals a tumor with sheets of tumor cell with similar morphology and immunohistochemistry profiles as those seen in peripheral blood and marrow. Abnormality of what gene is most likely associated with his condition?
A. BRAF
B. C-myc
C. MYD88
D. Notch 1
E. RUNX1


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Contents

Anemia

Lymphoid neoplasms