title: Transient myelofibrosis with autoimmune pancytopenia: a case report.
abstract: BACKGROUND
  Myelofibrosis associated with myelodysplasia is thought to herald poor prognosis in myelodysplastic syndrome (MDS).
  
  
  METHODS
  A 7-month-old boy presented with fever (39 degrees C), pancytopenia, and slight hepatosplenomegaly (3 and 2 cm, respectively). Bone marrow showed hypercellularity, hyperplasia of erythroblasts, and also myelofibrosis. IgG was 1,136 mg/dL, IgA was 131 mg/dL, and IgM was 89 mg/dL. Antinuclear and antineutrophil antibodies, red-blood-cell-associated IgG, antiplatelet antibodies, and Coombs test were positive. Karyotype was 46XY. No viral cause was evidenced. Mild myelodysplasia was revealed two months later, but was insufficient to support a diagnosis of MDS. The boy was treated with transfusion of packed cells, prednisolone 2 mg/kg/day for 3 weeks associated with intravenous gammaglobulin 400 mg/kg/day for 5 days. Direct Coombs remained positive 1 month after treatment for 5 months, myelofibrosis persisted for 3 months, and neutropenia for 21 months. After 3-year follow-up, hematological data were normal without any therapeutic intervention.
  
  
  CONCLUSIONS
  Myelofibrosis associated with mild myelodysplasia and pancytopenia can have a benign evolution in infants and young children.
authors.affiliation: Department of Pediatric Health, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Ibaraki, Japan. c0530319@kiban.md.tsukuba.ac.jp
authors.forename: Tomohei
authors.initials: T
authors.lastname: Nakao
authors.forename: Takashi
authors.initials: T
authors.lastname: Fukushima
authors.forename: Takashi
authors.initials: T
authors.lastname: Shimizu
authors.forename: Toru
authors.initials: T
authors.lastname: Nanmoku
authors.forename: Satoshi
authors.initials: S
authors.lastname: Fujiyama
authors.forename: Ryoko
authors.initials: R
authors.lastname: Nakajima
authors.forename: Fujiko
authors.initials: F
authors.lastname: Fukushima
authors.forename: Masayuki
authors.initials: M
authors.lastname: Noguchi
authors.forename: Ryo
authors.initials: R
authors.lastname: Sumazaki
mesh_terms: D001327:Autoimmune Diseases / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D007223:Infant; D007564:Japan; D008297:Male; D009196:Myeloproliferative Disorders / Q000175:diagnosis; D010198:Pancytopenia / Q000473:pathology; D055728:Primary Myelofibrosis / Q000473:pathology; D012075:Remission, Spontaneous