Clinical Pathology: General Principles, Hematology & Coagulation, Immunology & Histocompatibility, Urinalysis, Body Fluids, Clinical Microscopy, Genetic Testing

474) The bone marrow biopsy shown in Figures 1 and 2 is from a 68-year-old man with mild anemia (hemoglobin, 12 g/dL; reference range, 10.5 to 13.5 g/dL) with normal leukocyte (5.2 × 109/L; reference range, 6.0 to 11.0 × 109/L) and platelet counts (538 × 109/L, reference range, 130 to 400 × 109/L). Which one of the following is part of the major criteria for the diagnosis of primary myelofibrosis (PMF)?

• Primary myelofibrosis (PMF) is a myeloproliferative neoplasm and tends to affect patients in their sixth to seventh decade of life.

• Many patients with PMF are asymptomatic and present with splenomegaly or anemia, leukocytosis, and/or thrombocytosis.

• Early PMF may present with only thrombocytosis and must be distinguished from essential thrombocytosis (ET), another myeloproliferative neoplasm.

• To diagnosis PMF, all three major and two minor World Health Organization (WHO) criteria must be met. The major criteria are (1) megakaryocytic proliferation and atypia, (2) not meeting WHO criteria for other myeloid neoplasms, and (3) clonal molecular change or in the absence of a marker of clonality, exclusion of other causes of fibrosis. The minor criteria are (1) leukoerythroblastosis, (2) increase in serum lactate dehydrogenase (LDH), (3) anemia, and (4) splenomegaly.

• The most common clonal molecular alterations in PMF are Janus kinase 2 V617F (JAK2 V617F) and myeloproliferative leukemia virus W151L/K (MPL W151L/K).



 
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