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The Effect of Alfacalcidol in the Treatment of Idiopathic Myelofibrosis in Children

Journal of the Korean Pediatric Society 1994;37(3):339-346.
Published online March 15, 1994.
The Effect of Alfacalcidol in the Treatment of Idiopathic Myelofibrosis in Children
Soon Ki Kim1, Jeong Hee Kim1, Dae Hyun Lim1, Jong Woon Choi1, Byong Kwan Son1, Hyo Seop Ahn2
1Department of Pediatrics, College of Medicine, Inha University, Inha Hospital, Seongnam, Korea
2Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea
소아 특발성 골수섬유증의 치료에 있어서 알파칼시돌의 효과
김순기1, 김정희1, 임대현1, 최종운1, 손병관1, 안효섭2
1인하대학교 의과대학 소아과학교실
2서울대학교 의과대학 소아과학교실
Abstract
Idiopathic myelofibrosis (IMF), which is characterized by marrow fibrosis, leukoerythroblastic anemia, teardrop poikilocytosis and splenomegaly due to extramedullary hematopoiesis, has known to have no form of therapy. On the ground of the possibility of reversing collagen deposition in IMF using 1, 25-dihydroxycholecalciferol [1, 25(OH)2D3], we report here our observations of 5 patients (M:F = 1:4) with IMF before and after treatment with 0.5㎍/day of alfacalcidol, precursor of 1, 25(OH)2D3. In 3 of 5 patients the hemoglobin rose and in 4 of 5 the platelet count increased. Follow-up marrow examination revealed that marrow trephine reticulin fibrosis decreased according as the amelioration of clinical and laboratory findings. But these did not persist except one patient in spite of the sustained use of alfacalcidol. Our results suggest that alfacalcidol may have a therapeutic role in some patients with IMF. More extensive studies will be clarify the action of alfacalcidol in IMF.
Key Words: Idiopathic myelofibrosis children, 1, 25-dihydroxycholecalciferol, Alfacalcidol


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