Nakao Tomohei, Fukushima Takashi, Shimizu Takashi, Nanmoku Toru, Fujiyama Satoshi, Nakajima Ryoko, Fukushima Fujiko, Noguchi Masayuki, Sumazaki Ryo
Department of Pediatric Health, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Ibaraki, Japan.
Eur J Pediatr. 2009 Aug;168(8):1003-6. doi: 10.1007/s00431-008-0867-y. Epub 2008 Nov 6.
Myelofibrosis associated with myelodysplasia is thought to herald poor prognosis in myelodysplastic syndrome (MDS).
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.
Myelofibrosis associated with mild myelodysplasia and pancytopenia can have a benign evolution in infants and young children.
