Transient myelofibrosis with autoimmune pancytopenia: a case report.
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.
Nakao
Tomohei
T
Department of Pediatric Health, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Ibaraki, Japan. c0530319@kiban.md.tsukuba.ac.jp
Fukushima
Takashi
T
Shimizu
Takashi
T
Nanmoku
Toru
T
Fujiyama
Satoshi
S
Nakajima
Ryoko
R
Fukushima
Fujiko
F
Noguchi
Masayuki
M
Sumazaki
Ryo
R
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