All issues > Volume 37(3); 1994

Original Article

J Korean Pediatr Soc. 1994;37(3):339-346. Published online March 15, 1994.

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

Soon Ki SK Kim¹, Jeong Hee JH Kim¹, Dae Hyun DH Lim¹, Jong Woon JW Choi¹, Byong Kwan BK Son¹, Hyo Seop HS Ahn²

¹Department of Pediatrics, College of Medicine, Inha University, Inha Hospital, Seongnam, Korea
²Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea

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.

Keywords :Idiopathic myelofibrosis children, 1, 25-dihydroxycholecalciferol, Alfacalcidol