Wallach's Interpretation of Diagnostic Tests: Pathways to Arriving at a Clinical Diagnosis (354 page)

   Presence in the bone marrow biopsy of megakaryocytic proliferation and atypia, usually accompanied by either reticulin and/or collagen fibrosis; in the absence of significant reticulin fibrosis, the megakaryocyte changes must be accompanied by an increased bone marrow cellularity characterized by granulocytic proliferation and often decreased erythropoiesis (the prefibrotic cellular-phase disease).

   Not meeting WHO criteria for PV, CML, MDS, or other myeloid neoplasms.

   Demonstration of JAK2 V617F or other clonal markers such as MPL, or in the absence of a clonal marker, no evidence that the bone marrow fibrosis is due to an underlying inflammatory or other neoplastic diseases.

   
Minor criteria

   Leukoerythroblastosis

   Increased serum LDH

   Anemia

   Palpable splenomegaly

   Who Should Be Suspected of PMF?

   Patients with progressive splenomegaly reaching enormous size and resulting in hypersplenism as manifested by pancytopenia. Hepatomegaly may also be present.

   Patients aged >65 with constitutional symptoms: severe fatigue, symptoms due to enlarged spleen, weight loss, signs of a hypermetabolic state, pruritus, and pulmonary hypertension.

   Patients with progressive unexplained anemia with bizarre peripheral blood smear (PBS) morphology, and leukocytosis.

   Patients with thrombosis of splanchnic veins.