Transient myelofibrosis with autoimmune pancytopenia: a case report.
Eur J Pediatr
; 168(8): 1003-6, 2009 Aug.
Article
em En
| MEDLINE
| ID: mdl-18987883
ABSTRACT
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Pancitopenia
/
Doenças Autoimunes
/
Mielofibrose Primária
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Humans
/
Infant
/
Male
País como assunto:
Asia
Idioma:
En
Ano de publicação:
2009
Tipo de documento:
Article