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[自身免疫性溶血性贫血患者的长期观察]

[Long-term observation of patients with autoimmune hemolytic anemia].

作者信息

Kopeć I, Słomkowski M, Przybyszewska M, Szczepanik K, Maj S

机构信息

Klinika Hematologiczna Instytutu Hematologii, Warszawa.

出版信息

Acta Haematol Pol. 1991;22(1):58-68.

PMID:1823969
Abstract

Forty-nine cases of idiopathic and secondary autoimmune hemolytic anaemia (AIHA) were observed. Infections developed in 64% of them before the diagnosis of the idiopathic form had been established. In 83% of the patients warm antibodies were found, and in half these cases complement activation was observed on the blood cells. In 16% of cases autoantibodies to other tissues were present as well. Higher grade of anaemia and more severe course of the disease were observed in secondary AIHA. The survival time of patients with malignant lymphoma and haemolysis developing during the proliferation phase was twice as long as in cases with simultaneously diagnosed lymphoma and haemolysis. Idiopathic AIHA was more frequent chronic and mild. During over 2 years of follow-up haemolysis regressed in 44% of cases (in idiopathic AIHA in 65% and in secondary AIHA in 19%). The followed-up group comprised 36 patients. No immediate favourable effect of splenectomy was noted. In 43% of cases infections were a complication of pharmacological treatment and (or) splenectomy. Alloimmunization against blood cells was noted in 16% of cases.

摘要

观察了49例特发性和继发性自身免疫性溶血性贫血(AIHA)。其中64%在特发性形式的诊断确立之前就发生了感染。83%的患者发现有温抗体,其中半数病例在血细胞上观察到补体激活。16%的病例还存在针对其他组织的自身抗体。继发性AIHA患者贫血程度更高,病程更严重。恶性淋巴瘤患者在增殖期出现溶血,其生存时间是同时诊断淋巴瘤和溶血患者的两倍。特发性AIHA更常见为慢性且病情较轻。在超过2年的随访中,44%的病例溶血消退(特发性AIHA中为65%,继发性AIHA中为19%)。随访组包括36例患者。未观察到脾切除术有直接的良好效果。43%的病例中感染是药物治疗和(或)脾切除术的并发症。16%的病例出现了针对血细胞的同种免疫。

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