Practice questions myeloid neoplasms 1

Practice questions
Myeloid neoplasms I

© Jun Wang, MD, PhD

1. Use this case for the next two questions. A 51-year-old man presents with gum bleeding for 2 days. He has had purpura on his back and legs for the last two weeks. Physical examination reveals multiple petechiae on his oral mucosa, and multiple purpura measuring up to 3 cm in greatest dimension on his back and legs. No lymphadenopathy nor organomegaly are identified. Laboratory studies reveal a hemoglobin at 8.5 g/dL (normal 13-18g/dL), white cell count at 49 x 109/L (normal 5-11 x 109/L), platelet count at 7 x 109/L (normal 150-450 x 109/L). Peripheral blood smears reveal 25% of white cells are myeloblasts, monoblasts and promonocytes. Bone marrow aspiration and biopsy reveal large amount of blasts with similar morphology as those seen in peripheral blood. Eosinophil precursors with basophilic granules are seen. In addition, abnormal erythroid precursors with nuclear budding are seen. Karyotype analysis reveals inv(16)(p13.1;q22). What is the most likely diagnosis?
A. Acute lymphoblastic leukemia
B. Acute myeloid leukemia
C. Chronic myeloid leukemia
D. Chronic myelomonocytic leukemia
E. Myelodysplastic syndrome

2. A 51-year-old man presents with gum bleeding for 2 days. He has had purpura on his back and legs for the last two weeks. Physical examination reveals multiple petechiae on his oral mucosa, and multiple purpura measuring up to 3 cm in greatest dimension on his back and legs. No lymphadenopathy nor organomegaly are identified. Laboratory studies reveal a hemoglobin at 8.5 g/dL (normal 13-18g/dL), white cell count at 49 x 109/L (normal 5-11 x 109/L), platelet count at 7 x 109/L (normal 150-450 x 109/L). Peripheral blood smears reveal 25% of white cells are myeloblasts, monoblasts and promonocytes. Bone marrow aspiration and biopsy reveal large amount of blasts with similar morphology as those seen in peripheral blood. Eosinophil precursors with basophilic granules are seen are seen. In addition, abnormal erythroid precursors with nuclear budding. Karyotype analysis reveals inv(16)(p13.1;q22). Abnormality of what gene is most likely associated with his condition?
A. ABL
B. MYH11
C. MYD 88
D. RAR alpha
E. RUNX1

3. Use this case for the next three questions. A 32-year-old woman presents with exaggerated fatigue and gingival bleeding for three days. Her past medical history is unremarkable. Physical examination reveals pale skin and mucosa, as well as multiple petechiae in this oral mucosa and conjunctiva. Multiple purpura measuring up to 4 cm in greatest dimension are seen on her back and arms. No lymphadenopathy nor organomegaly are seen. Laboratory studies reveal a hemoglobin at 8 g/L (normal 12-16 g/dL), white count 17 x 109/L (normal 5-11 x 109/L), platelet count at 85 x 109/L (normal 150-450 x 109/L). WBC differential reveals 20% lymphocytes, 10% monocytes, 5% eosinophils and 65% atypical cells. These atypical cells have moderate amount of cytoplasm containing various amount of azurophilic needle shaped materials. Morphology of red blood cells and platelets are unremarkable. What is the most likely diagnosis?
A. Acute lymphoblastic leukemia
B. Acute promyelocytic leukemia
C. Chronic myelogenous leukemia
D. Chronic myelomonocytic leukemia
E. Immune thrombocytopenic purpura

4. A 32-year-old woman presents with exaggerated fatigue and gingival bleeding for three days. Her past medical history is unremarkable. Physical examination reveals pale skin and mucosa, as well as multiple petechiae in this oral mucosa and conjunctiva. Multiple purpura measuring up to 4 cm in greatest dimension are seen on her back and arms. No lymphadenopathy nor organomegaly are seen. Laboratory studies reveal a hemoglobin at 8 g/L (normal 12-16 g/dL), white count 17 x 109/L (normal 5-11 x 109/L), platelet count at 85 x 109/L (normal 150-450 x 109/L). WBC differential reveals 20% lymphocytes, 10% monocytes, 5% eosinophils and 65% atypical cells. These atypical cells have moderate amount of cytoplasm containing various amount of azurophilic needle shaped materials, . Morphology of red blood cells and platelets are unremarkable. Abnormality of what gene is most likely found in these atypical cells?
A. ABL
B. ADAMTS13
C. MYH11
D. RAR alpha
E. RUNX1

5. A 32-year-old woman presents with exaggerated fatigue and gingival bleeding for three days. Her past medical history is unremarkable. Physical examination reveals pale skin and mucosa, as well as multiple petechiae in this oral mucosa and conjunctiva. Multiple purpura measuring up to 4 cm in greatest dimension are seen on her back and arms. No lymphadenopathy nor organomegaly are seen. Laboratory studies reveal a hemoglobin at 8 g/L (normal 12-16 g/dL), white count 17 x 109/L (normal 5-11 x 109/L), platelet count at 85 x 109/L (normal 150-450 x 109/L). WBC differential reveals 20% lymphocytes, 10% monocytes, 5% eosinophils and 65% atypical cells. These atypical cells have moderate amount of cytoplasm containing various amount of azurophilic needle shaped materials, some forming a bundle like arrangement. Morphology of red blood cells and platelets are unremarkable. What is a life threatening complication for this patient?
A. Diffuse intravascular coagulation
B. End stage renal disease
C. Myelofibrosis
D. Rupture of massively enlarged spleen
E. Severe anemia

6. Use this case for the next two questions. A 75-year-old man presents with fatigue and occasional dizziness for 6 months. He has a history of colon adenocarcinoma that was treated with surgery and chemotherapy 10 years ago. Physical examination reveal pallor skin, but no other significant abnormalities are seen. Laboratory studies reveal a hemoglobin of 10.2 g/dL (normal 13-18 g/dL), WBC at 4 x 109/L (normal 5-11 x 109/L), platelet 110 x 109/L (normal 150-450 x 109/L), MCV 120 fL (normal 78-100 fL) and a red cell distribution width (RDW) at 15.5 % (normal 11-14%). White cell differential is within normal range. Peripheral blood smears reveal marked variation in the sizes of red cells. A few bilobed neutrophils are seen. Colonoscopic examination reveal no evidence of tumor. Bone marrow core biopsy and aspiration reveal a hypercellular marrow. Some erythroid precursors with nuclear budding. There are hypolobulated megakaryocytes. Flow cytometry studies reveal a less than 5% myeloblast population. What additional laboratory test is most likely suggestive of the diagnosis?
A. Autoantibody screening
B. Cytogenetic and molecular studies
C. Hemoglobin electrophoresis
D. Reticulocytes count
E. Serum iron and ferritin test

7. A 75-year-old man presents with fatigue and occasional dizziness for 6 months. He has a history of colon adenocarcinoma that was treated with surgery and chemotherapy 10 years ago. Physical examination reveal pallor skin, but no other significant abnormalities are seen. Laboratory studies reveal a hemoglobin of 10.2 g/dL (normal 13-18 g/dL), WBC at 4 x 109/L (normal 5-11 x 109/L), platelet 110 x 109/L (normal 150-450 x 109/L), MCV 120 fL (normal 78-100 fL) and a red cell distribution width (RDW) at 15.5 % (normal 11-14%). White cell differential is within normal range. Peripheral blood smears reveal marked variation in the sizes of red cells. A few bilobed neutrophils are seen. Colonoscopic examination reveal no evidence of tumor. Bone marrow core biopsy and aspiration reveal a hypercellular marrow. Some erythroid precursors with nuclear budding. There are hypolobulated megakaryocytes. Flow cytometry studies reveal a less than 5% myeloblast population. What is most likely diagnosis?
A. Acute myelogenous leukemia
B. Chronic myelogenous leukemia
C. Iron deficiency anemia
D. Myelodysplastic syndrome
E. Thalassemia

8. Use this case for the next three questions. A 33-year-old woman presents with fatigue and left upper abdomen discomfort for 6 months. She has a history of gastric peptic ulcer, helicobacter pylori-associated chronic gastritis 5 years ago. Physical examination reveals a non tender spleen at approximately 10 cm below the left costal margin. No hepatomegaly nor lymphadenopathy are seen. Laboratory studies reveal a hemoglobin of 8.3 g/dL (normal 12-16 g/dL), WBC 130 x 109/L, and platelets 600 x 109/L (normal 150-450 x 109/L). WBC differential reveal mature neutrophils (50%), bands (15%), metamyelocytes (7%), myelocytes (5%), myeloblast (2%), monocytes (3%), lymphocytes (11%), eosinophils (2%) and basophils (5%). Bone marrow biopsy and aspiration reveal markedly hypercellular marrow with hyperplasia and normal maturation in all lineages. No lymphocytes population are seen. There is no evidence of abnormal morphology of hematopoietic precursors. No fibrotic changes are seen. Flowcytometry studies of marrow reveal a myeloblast population of 5% of the cellularity. What laboratory test would most likely be abnormal?
A. Blood JAK2V617F screen for mutation
B. Cytogenetic/FISH studies for chromosome translocation
C. Immunofixation for monoclonal gammopathy
D. Molecular studies for neoplastic cell human T cell leukemia virus

9. A 33-year-old woman presents with fatigue and left upper abdomen discomfort for 6 months. She has a history of gastric peptic ulcer, helicobacter pylori-associated chronic gastritis 5 years ago. Physical examination reveals a non tender spleen at approximately 10 cm below the left costal margin. No hepatomegaly nor lymphadenopathy are seen. Laboratory studies reveal a hemoglobin of 8.3 g/dL (normal 12-16 g/dL), WBC 130 x 109/L, and platelets 600 x 109/L (normal 150-450 x 109/L). WBC differential reveal mature neutrophils (50%), bands (15%), metamyelocytes (7%), myelocytes (5%), myeloblast (2%), monocytes (3%), lymphocytes (11%), eosinophils (2%) and basophils (5%). Bone marrow biopsy and aspiration reveal markedly hypercellular marrow with hyperplasia and normal maturation in all lineages. No lymphocytes population are seen. There is no evidence of abnormal morphology of hematopoietic precursors. No fibrotic changes are seen. Flowcytometry studies of marrow reveal a myeloblast population of 5% of the cellularity. What is the most likely diagnosis?
A. Acute myelogenous leukemia
B. Chronic myelogenous leukemia
C. Chronic myelomonocytic leukemia
D. Myelodysplastic syndrome
E. Thalassemia

10. A 33-year-old woman presents with fatigue and left upper abdomen discomfort for 6 months. She has a history of gastric peptic ulcer, helicobacter pylori-associated chronic gastritis 5 years ago. Physical examination reveals a non tender spleen at approximately 10 cm below the left costal margin. No hepatomegaly nor lymphadenopathy are seen. Laboratory studies reveal a hemoglobin of 8.3 g/dL (normal 12-16 g/dL), WBC 130 x 109/L, and platelets 600 x 109/L (normal 150-450 x 109/L). WBC differential reveal mature neutrophils (50%), bands (15%), metamyelocytes (7%), myelocytes (5%), myeloblast (2%), monocytes (3%), lymphocytes (11%), eosinophils (2%) and basophils (5%). Bone marrow biopsy and aspiration reveal markedly hypercellular marrow with hyperplasia and normal maturation in all lineages. No lymphocytes population are seen. There is no evidence of abnormal morphology of hematopoietic precursors. No fibrotic changes are seen. Flowcytometry studies of marrow reveal a myeloblast population of 5% of the cellularity. Abnormality of what gene is most likely associated with these findings?
A. BCL2
B. BCR
C. CALR
D. JAK2
E. MPL

11. Use this case for the next three questions. A 62-year-old male farmer presents with fatigue and skin itching for 3 year. He developed burning upper stomachache in recent 3 months. His past medical history is unremarkable. He smokes cigarette one pack per day for 45 years. Physical examination reveals reveals a ruddy complexion but the skin is otherwise unremarkable. Spleen is 5 cm below left costal margin and non tender. Gastroscopic examination performed 1 month ago reveals a 1 cm ulcer at his antrum. Biopsy of the ulcer reveal ulcerated gastric mucosa with diffuse neutrophilic and lymphoplasmacytic infiltrate. No helicobacter pylori is identified. Immunohistochemistry studies reveal mixed lymphocytic population without evidence of light chain restriction. Current laboratory tests reveal a hemoglobin at 23.5 g/dL (normal 14-18 g/dL), white blood cell count at 8.5 x 109/L (normal 4.5-11 x 109/L) with normal differential, platelets at 476 x 109/L (normal 150-450 x 109/L). Peripheral blood smears reveal some red blood cells have enlarged central pallor areas. His ferritin is 11 mg/ml (normal 26-388 mg/ml), iron 45 microgram/dL (normal 65-175 microgram/dL), iron saturation rate 12% (normal 22-55%). Bone marrow biopsy reveal a hypercellular marrow with erythroid hyperplasia. Megakaryocytic components are slightly increased. No atypical hematopoietic precursors nor abnormal lymphocytic aggregates are seen. What is most likely the diagnosis?
A. Essential thrombocythemia
B. Iron deficiency anemia
C. Marginal zone lymphoma with marrow involvement
D. Myelodysplastic syndrome
E. Polycythemia vera

12. A 62-year-old male farmer presents with fatigue and skin itching for 3 year. He developed burning upper stomachache in recent 3 months. His past medical history is unremarkable. He smokes cigarette one pack per day for 45 years. Physical examination reveals reveals a ruddy complexion but the skin is otherwise unremarkable. Spleen is 5 cm below left costal margin and non tender. Gastroscopic examination performed 1 month ago reveals a 1 cm ulcer at his antrum. Biopsy of the ulcer reveal ulcerated gastric mucosa with diffuse neutrophilic and lymphoplasmacytic infiltrate. No helicobacter pylori is identified. Immunohistochemistry studies reveal mixed lymphocytic population without evidence of light chain restriction. Current laboratory tests reveal a hemoglobin at 23.5 g/dL (normal 14-18 g/dL), white blood cell count at 8.5 x 109/L (normal 4.5-11 x 109/L) with normal differential, platelets at 476 x 109/L (normal 150-450 x 109/L). Peripheral blood smears reveal some red blood cells have enlarged central pallor areas. His ferritin is 11 mg/ml (normal 26-388 mg/ml), iron 45 microgram/dL (normal 65-175 microgram/dL), iron saturation rate 12% (normal 22-55%). Bone marrow biopsy reveal a hypercellular marrow with erythroid hyperplasia. Megakaryocytic components are slightly increased. No atypical hematopoietic precursors nor abnormal lymphocytic aggregates are seen. What additional test is most likely abnormal?
A. Blood JAK2V617F erythropoietin screen for mutation
B. Cytogenetic/FISH studies for chromosome translocation
C. Immunofixation for monoclonal gammopathy
D. Molecular studies for neoplastic cell human T cell leukemia virus

13. A 62-year-old male farmer presents with fatigue and skin itching for 3 year. He developed burning upper stomachache in recent 3 months. His past medical history is unremarkable. He smokes cigarette one pack per day for 45 years. Physical examination reveals reveals a ruddy complexion but the skin is otherwise unremarkable. Spleen is 5 cm below left costal margin and non tender. Gastroscopic examination performed 1 month ago reveals a 1 cm ulcer at his antrum. Biopsy of the ulcer reveal ulcerated gastric mucosa with diffuse neutrophilic and lymphoplasmacytic infiltrate. No helicobacter pylori is identified. Immunohistochemistry studies reveal mixed lymphocytic population without evidence of light chain restriction. Current laboratory tests reveal a hemoglobin at 23.5 g/dL (normal 14-18 g/dL), white blood cell count at 8.5 x 109/L (normal 4.5-11 x 109/L) with normal differential, platelets at 476 x 109/L (normal 150-450 x 109/L). Peripheral blood smears reveal some red blood cells have enlarged central pallor areas. His ferritin is 11 mg/ml (normal 26-388 mg/ml), iron 45 microgram/dL (normal 65-175 microgram/dL), iron saturation rate 12% (normal 22-55%). Bone marrow biopsy reveal a hypercellular marrow with erythroid hyperplasia. Megakaryocytic components are slightly increased. No atypical hematopoietic precursors nor abnormal lymphocytic aggregates are seen. Abnormality of what gene is most likely associated with his condition?
A. ABL
B. API2
C. IgH
D. JAK2
E. MYD88

14. Use this case for the next three questions. A 61-year-old woman presents with headache and dizziness for 6 months. A single dose of aspirin may relieve her headache for a few days. Her past history is unremarkable. Physical examination is unremarkable. No significant neurological abnormality is seen. Laboratory studies reveal a hemoglobin at 14.3 g/dL (normal 12-16 g/dL), white cell count at 6.7 x 109/L (normal 4.5-11 x 109/L) with normal differential, platelet counts at 852 x 109/L (normal 150-450 x 109/L). Peripheral blood smear reveals increased number of platelets. Giant platelets are occasionally seen. No significant morphological abnormality is presents in red cell and white cells. Bone marrow biopsy reveal a slightly hypercellular marrow with megakaryotic hyperplasia. Small clusters of megakaryocytes are present. No abnormal lymphocytic population is seen. What additional study is most likely abnormal?
A. Blood JAK2V617F erythropoietin screen for mutation
B. Cytogenetic/FISH studies for chromosome translocation
C. Head CT for brain mass
D. Immunofixation for monoclonal gammopathy
E. Molecular studies for neoplastic cell human T cell leukemia virus

15. A 61-year-old woman presents with headache and dizziness for 6 months. A single dose of aspirin may relieve her headache for a few days. Her past history is unremarkable. Physical examination is unremarkable. No significant neurological abnormality is seen. Laboratory studies reveal a hemoglobin at 14.3 g/dL (normal 12-16 g/dL), white cell count at 6.7 x 109/L (normal 4.5-11 x 109/L) with normal differential, platelet counts at 852 x 109/L (normal 150-450 x 109/L). Peripheral blood smear reveals increased number of platelets. Giant platelets are occasionally seen. No significant morphological abnormality is presents in red cell and white cells. Bone marrow biopsy reveal a slightly hypercellular marrow with megakaryotic hyperplasia. Small clusters of megakaryocytes are present. No abnormal lymphocytic population is seen. What is most likely the diagnosis?
A. Brain tumor
B. Chronic myelogenous leukemia
C. Essential thrombocythemia
D. Myelodysplastic syndrome
E. Polycythemia vera

16. A 61-year-old woman presents with headache and dizziness for 6 months. A single dose of aspirin may relieve her headache for a few days. Her past history is unremarkable. Physical examination is unremarkable. No significant neurological abnormality is seen. Laboratory studies reveal a hemoglobin at 14.3 g/dL (normal 12-16 g/dL), white cell count at 6.7 x 109/L (normal 4.5-11 x 109/L) with normal differential, platelet counts at 852 x 109/L (normal 150-450 x 109/L). Peripheral blood smear reveals increased number of platelets. Giant platelets are occasionally seen. No significant morphological abnormality is presents in red cell and white cells. Bone marrow biopsy reveal a slightly hypercellular marrow with megakaryotic hyperplasia. Small clusters of megakaryocytes are present. No abnormal lymphocytic population is seen. Abnormality of what gene is most likely associated with her condition?
A. ABL
B. BRAF
C. C-MYC
D. JAK2
E. RAR-alpha


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