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本文报道一例急性骨髓纤维化(AMF),男、52岁,严重贫血(Hb19g/L)经多种升血药物、胎肝细胞悬液输注等治疗20个月无明显好转;靠输血维持生命。经肌注维生素D_3(VD_3)后两个月病情显著缓解,不需输血,Hb稳步升至93g/L出院。随访9个月生活自理,Hb114g/L,髓象及骨髓活检均明显好转,无副反应。VD_3治疗AMF是一新进展,此病人是国内取得临床成功的首例。据文献证明VD_3在体内羟化为1,25(OH)_2D_3活性物质能抑制异常巨核细胞增生,减少促进MF的PDGF,并诱导髓细胞向单核细胞、臣噬细胞和粒细胞方向成熟,释放胶原酶促进胶原裂解,因而MF改善。
This article reports a case of acute myelofibrosis (AMF), male, 52 years old, severe anemia (Hb19g / L) by a variety of blood-lifting drugs, fetal liver cell suspension infusion therapy for 20 months without significant improvement; . After intramuscular injection of vitamin D_3 (VD_3) two months after the disease was significantly relieved, without blood transfusion, Hb steadily rose to 93g / L discharged. Follow-up 9 months living self-care, Hb114g / L, medullary and bone marrow biopsy were significantly improved, no side effects. VD_3 treatment of AMF is a new progress, this patient is the first case of clinical success in China. According to the literature, hydroxylation of VD_3 to 1,25 (OH) _2D_3 in vivo inhibits the proliferation of abnormal megakaryocytes, decreases the promotion of PDGF in MF, and induces the maturation and release of myeloid cells toward monocytes, granulocytes, and granulocytes Collagenase promotes collagen cleavage and thus MF improves.