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[原发性骨髓纤维化]

[Idiopathic myelofibrosis].

作者信息

Saad G M, de Almeida T V

出版信息

Rev Paul Med. 1991 Mar-Apr;109(2):47-50.

PMID:1887177
Abstract

The authors make a review of recent data in the literature and compare them to their own cases between 1983 and 1988. They analyzed eight patients with idiopathic myelofibrosis confirmed with bone marrow biopsy of the posterior iliac wrist with Jamshidi's needle. Most patients were between 55 and 60 years old (5 male and 3 female). Major symptoms were weakness and bleeding (6/8 cases), weight loss and bone distress (4/8 cases). In general, physical signs were splenomegaly and anemia (7/8 cases), hepatomegaly (5/8 cases), and jaundice (2/8 cases). Laboratory features were variable. Most cases were diagnosed in an advanced stage of the disease. Therapy with busulfan, prednisone, oxymetholone and radiotherapy of the spleen was used alone or in combination to relieve compressive abdominal symptoms. This review shows that idiopathic myelofibrosis should be included in the differential diagnosis of patients with hepatosplenomegaly and anemia.

摘要

作者对文献中的近期数据进行了综述,并将其与1983年至1988年间他们自己的病例进行了比较。他们分析了8例经Jamshidi针行髂后上棘骨髓活检确诊的特发性骨髓纤维化患者。大多数患者年龄在55至60岁之间(5例男性,3例女性)。主要症状为虚弱和出血(6/8例)、体重减轻和骨痛(4/8例)。一般来说,体征为脾肿大和贫血(7/8例)、肝肿大(5/8例)和黄疸(2/8例)。实验室检查结果各不相同。大多数病例在疾病晚期被诊断出来。使用白消安、泼尼松、羟甲烯龙治疗以及脾脏放疗单独或联合使用以缓解腹部压迫症状。该综述表明,特发性骨髓纤维化应纳入肝脾肿大和贫血患者的鉴别诊断中。

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