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Transient myelofibrosis with autoimmune pancytopenia: a case report
Tomohei Nakao1, Takashi Fukushima, Takashi Shimizu
1Department of Pediatric Health, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Ibaraki, Japan. c0530319@kiban.md.tsukuba.ac.jp
Introduction:
Myelofibrosis associated with myelodysplasia is thought to herald poor prognosis in myelodysplastic syndrome (MDS).
Case Report:
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.
Conclusion:
Myelofibrosis associated with mild myelodysplasia and pancytopenia can have a benign evolution in infants and young children.
