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[α干扰素治疗特发性骨髓纤维化]

[Alpha interferon treatment of idiopathic myelofibrosis].

作者信息

Cervantes F, Alcorta I, Escoda L, Montserrat E, Rozman C

机构信息

Escuela de Hematología Farreras Valentí, Hospital Clínic i Provincial, Universidad de Barcelona.

出版信息

Med Clin (Barc). 1993 Oct 23;101(13):498-500.

PMID:8231385
Abstract

Idiopathic myelofibrosis is a chronic myeloproliferative syndrome for which there is no effective treatment. The good results obtained with interferon in other chronic myeloproliferative syndromes have led their being tested in idiopathic myelofibrosis, but to date the experience is scarce. Four patients out of a total of 12 diagnosed with idiopathic myelofibrosis over a period of 3 years were selected for interferon treatment. Patients with low leukocyte or platelet counts or with contraindication for administration of the drug were excluded. Alpha-2b interferon was administered at an initial dose of 3 MU/day which was increased at 4-6 weeks to 5 MU/day in cases of insufficient response and if tolerance so permitted. In patients in whom favorable response was observed a maintenance schedule was initiated with low doses of interferon. Treatment was discontinued in two patients due to bad tolerance at 6 and 8 weeks of initiation of treatment with no response having been observed until that time. In the other patients favorable response was reported to interferon after 5 months of treatment with disappearance of the symptomatology, normalization of the hemo-peripheral values and a marked reduction of splenomegaly. This responses was accompanied by a decrease in bone marrow fibrosis in one case and total disappearance of the same in the other patient. Alpha-2b interferon constitutes an effective therapy for a selected number of patients with idiopathic myelofibrosis. Greater experience would allow the identification of the subgroup of patients who may benefit from this type of treatment.

摘要

特发性骨髓纤维化是一种慢性骨髓增殖性综合征,目前尚无有效治疗方法。干扰素在其他慢性骨髓增殖性综合征中取得的良好疗效促使其在特发性骨髓纤维化中进行试验,但迄今为止相关经验较少。在3年期间确诊的12例特发性骨髓纤维化患者中,有4例被选用于干扰素治疗。排除白细胞或血小板计数低或有药物使用禁忌证的患者。α-2b干扰素初始剂量为3MU/天,若反应不足且耐受允许,在4 - 6周时增至5MU/天。对观察到有良好反应的患者开始采用低剂量干扰素维持治疗。2例患者在开始治疗6周和8周时因耐受性差而停药,在此之前未观察到反应。其他患者在治疗5个月后报告对干扰素有良好反应,症状消失,血液外周指标恢复正常,脾肿大明显减轻。1例患者骨髓纤维化减轻,另1例患者骨髓纤维化完全消失。α-2b干扰素对部分特发性骨髓纤维化患者构成有效治疗方法。更多的经验将有助于确定可能从这类治疗中获益的患者亚组。

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