Practice questions answers myeloid neoplasms

Practice questions answers
Myeloid neoplasms I
©Jun Wang, MD, PhD


1. B. For acute myeloid leukemia, a diagnosis can be made with either >20% blasts, or confirmed recurrent genetic abnormalities. Acute lymphoblastic leukemia has LYMPHOBLAST, not myeloblast. Chronic myelogenous leukemia has less than 20% blasts and 9;22 translocation, Philadelphia chromosome, involving Abl and Bcr. Chronic myelomonocytic leukemia may have increased myeloblast, monocytes and monoblast, but the number of blasts is less than 20%, and inv(16)(p13.1;q22) is not seen in CMML. The presence of abnormal erythroid precursors are suggestive of myelodysplastic syndrome, however, by definition, if the quantity of blasts > 20%, or with confirmation of associated recurrent genetic abnormalities, the diagnosis is acute myeloid leukemia.


2. B. Abl is associated with chronic myelogenous leukemia, MTD 88 with lymphoplasmacytic lymphoma, RAR alpha with APL with PML-RARA, RUNX1-RUNX1T1 with AML with t(8;21)(q22;q22.1).


3. B. Presence of cytopenia and faggot cells is most consistent with M3. Acute lymphoblastic leukemia lymphoblasts do not have Auer rods. Thrombocytopenia is seen in ITP, but the presence of large amount of promyelocytes and faggot cells is usually absent in ITP.

4. D. APL with PML-RARA involves retinoid acid receptor-alpha (RAR-alpha). ABL abnormality is seen in chronic myelogenous leukemia, ADAMTS13 in thrombotic thrombocytopenic purpura, MYH11 in AML with inv(16)(p13.1q22), RUNX1-RUNX1T1 with AML with t(8;21)(q22;q22.1).

5. A. For patient with APL with PML-RARA, disseminated intravascular coagulation is not uncommon before and during chemotherapy. End stage renal disease may be seen in association with multiple myeloma and other plasma cell neoplasms. Myelofibrosis and massive splenomegaly usually occurs in myeloproleferative neoplasms.

6.  B. The presences of cytopenia in peripheral blood but hypercellularity in marrow, as well as the presence of dysplastic appearing hematopoietic precursors is consistent with myelodysplastic syndrome. Cytogenetics and molecular studies aids in further classification and determining prognostic risk group and therapy, as well as ruling out acute myeloid leukemia. Autoantibody screening is useful for autoimmune hemolytic anemia, hemoglobin electrophoresis for hemoglobinopathies, reticulocytes count in hemolytic anemia, iron and ferritin for iron deficiency anemia.

7. D. Peripheral cytopenia with hypercellular marrow and dysplastic changes is consistent with myelodysplastic syndrome (MDS), if the blast components are less than 20%, or no specific genetic abnormality is detected. Acute myeloid leukemia has more than 20% blasts. Chronic myelogenous leukemia is one of the myeloproleferative neoplasms, and dysplasia is not seen. Iron deficiency anemia and thalassemia do not have dysplastic changes.

8. B. High white cell count and hypercellular marrow with normal maturation and without dysplasia is consistent with myeloproleferative neoplasms. Thrombocytosis can be seen in chronic myelogenous leukemia, essential thrombocythemia and polycythemia vera. However, one of the four diagnostic criteria of ET is NO or little granulocytic or erythroid proliferation, not likely this patient that all lineages are involved. Anemia in this patient ruled out polycythemia vera. Immunofixation is used to detect monoclonal gammopathy associated with plasma neoplasms. There is no indications of plasma cell abnormality in this patient. No lymphocytic abnormality is seen, so there is no indication for any tests for adult T-cell leukemia/lymphoma. JAK2V617F erythropoietin screening is indicated for patients with features of polycythemia vera, while JAK2V617F screening is used in patients suspicious of having essential thrombocythemia.

9. B. See discussion for question 7.

10. B. The two genes involved in 9;22 translocation are abl and bcr. Bcl2 abnormality is seen in follicular lymphoma, CALR, JAK2 and MPL in essential thrombocythemia and primary myelofibrosis. JAK2 abnormality can also be seen in polycythemia vera.

11. E. High level of red blood cells and thrombocytosis are consistent with polycythemia vera. The clinical symptoms are associated with increased blood volume, red cell mass, distorted vessels and impaired oxygen delivery due to sludging of blood. Peptic ulcer is associated with increased histamine levels released by basophils. Essential thrombocythemia by definition should not have erythroid hyperplasia. Gastric ulcer may be a presentation of marginal zone lymphoma, but there is no evidence of monoclonal lymphocytic infiltrate (light chain restriction). The morphological abnormalities of red cells (enlarged central pallor area) and laboratory result is due to relative iron deficiency.

12. A. See discussion of question 8.

13. D. JAK2 mutation can be seen in essential thrombocythemia, polycythemia vera and primary myelofibrosis. Abl abnormality is seen in chronic myelogenous leukemia, API2 in marginal zone lymphoma, IgH in plasma cell neoplasms, including multiple myeloma, MYD 88 in lymphoplasmacytic lymphoma.

14. A. Many patients with essential thrombocythemia are asymptomatic. However, headache is the most common presentation in those with symptoms. Isolated thrombocytosis and marrow megakaryocytic hyperplasia are consistent with essential thrombocythemia. Intracranial mass may cause headache, but usually have other neurological abnormalities. Also see discussion of question 8.

15. C. Diagnosis of essential thrombocythemia is based on solitary thrombocytosis with solitary megakaryocytic hyperplasia in marrow. Also see discussion of question 8.

16. D. Abnormality of abl is seen in chronic myelogenous leukemia, BRAF in hairy cell leukemia, c-myc in Burkitt lymphoma, and RAR-alpha in APL with PML-RARA.

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Anemia

Lymphoid neoplasms