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KMID : 0384119960160030301
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Korean Journal of Clinical Pathology
1996 Volume.16 No. 3 p.301 ~ p.306
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Resolution of Idiopathic Myelofibrosis in an Infant
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최성숙
이효원/김은영/윤혜령/강명서/김진주
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Abstract
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Idiopathic myelofibrosis is a chronic myeloproliferative disorder characterized by fibrosis of the bone marrow accompanied by leukoerythroblastosis of peripheral blood and splenomegaly with extramedullary hematopoiesis without associated primary
condition. Idiopathic myelofibrosis is thought to be due to clonal proliferation of abnormal pluripotent stem cell with a secondary and presumptive reactive fibrosis of the bone marrow. It is a disease of adult and rare in children. The
myelofibrosis in
children usually follows a fulminant course, with death often occuring in the first year.
We experienced a case of idiopathic myelofibrosis in a 3 month-old girl with poor weight gain. Hepatomegaly was found. Bone marrow biopsy revealed myelofibrosis. The underlying diseases of myelofibrosis had not been detected. We report a case of
idiopathic myelofibrosis in an infant in whom resolution occurred after 14 months later with alphacalcidol treatment(Korean J Clin Pathol 1996;16(3):301~6).
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