title: Is there a role for the use of IFN-α in primary myelofibrosis?
abstract: A 62-year-old man with a history of diabetes and hypertension is referred to your hematology clinic for an incidental discovery of anemia. He does not have any constitutional symptoms and previous blood counts have been within the normal range. He has hepatosplenomegaly with a palpable spleen of 6 cm below the left costal margin and a liver size of 2.5 cm below the right costal margin. Laboratory evaluation shows a WBC count of 12.8 K/μL, hemoglobin of 11.0 g/dL, and platelets of 202 K/μL, with a mean corpuscular volume of 85.7, 72% neutrophils, 13% lymphocytes, 4% monocytes, 5% eosinophils, 1% basophils, 1% promyelocytes, 4% myelocytes, and lactate dehydrogenase of 447 U/L (upper limit of normal is < 340 U/L). Peripheral blood smear shows 2+ teardrop-shaped RBCs, large hypogranular platelets, and rare nucleated RBCs. Bone marrow (BM) biopsy exhibits a hypercellular BM with atypical megakaryocytes and increased reticulin fibrosis (MF-1). BCR-ABL gene rearrangement by FISH was negative and JAK2 V617F mutation was 95% positive. He was diagnosed with primary myelofibrosis considered low risk (risk score of 0) by the International Prognostic Scoring System.(1) Because he is low risk and asymptomatic, he does not need treatment at this time.(2) However, he has read about the possible clinical benefits of IFN-α and its potential reduction of BM fibrosis and wonders whether this would be an appropriate treatment.
authors.affiliation: Stanford University School of Medicine, Stanford, CA 94305, USA.
authors.forename: Huong Marie
authors.initials: HM
authors.lastname: Nguyen
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Kiladjian
mesh_terms: D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D001854:Bone Marrow Cells / Q000166:cytology; D002986:Clinical Trials as Topic; D006405:Hematology / Q000379:methods; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D016898:Interferon-alpha / Q000378:metabolism*; D053614:Janus Kinase 2 / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009154:Mutation; D055728:Primary Myelofibrosis / Q000235:genetics* / Q000378:metabolism*
chemical_list: D016898:Interferon-alpha; C507924:JAK2 protein, human; D053614:Janus Kinase 2