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三名患有慢性获得性纯红细胞再生障碍性贫血的家族性患者

[Three familial patients with chronic acquired pure red cell aplasia].

作者信息

Yamazaki T, Yoshizawa T, Kubo Y, Sawada U, Horie T, Adachi Y, Takasago M

机构信息

First Department of Internal Medicin, Nihon University.

出版信息

Rinsho Ketsueki. 1992 Jul;33(7):975-80.

PMID:1507420
Abstract

It has not been reported that chronic acquired pure red cell aplasia (PRCA) occurs among family members. The authors report his at familial patients with chronic acquired PRCA, a 23-year-old male, a 54-old uncle and a 20-year-old male cousin. They had no complication sor thymoma, and hematological findings were compatible with typical chronic acquired PRCA. In the 23-year-old male, the autologous BFU-E number was suppressed by the addition of pretreatment serum, and in the his uncle, the autologous CFU-E number was suppressed by the addition of IgG fraction of plasma. In these cases, the development of PRCA was considered to be based on the autoimmune mechanism due to by the presence of serum antibodies. This suggests the possibility that genetically inherited susceptibility may also be an important factor causing chronic acquired PRCA.

摘要

尚未有家庭成员间发生慢性获得性纯红细胞再生障碍性贫血(PRCA)的报道。作者报告了3例家族性慢性获得性PRCA患者,分别为一名23岁男性、一名54岁的叔叔和一名20岁的男性表弟。他们均无胸腺瘤并发症,血液学检查结果与典型的慢性获得性PRCA相符。在这名23岁男性患者中,加入预处理血清后自体爆式红系集落形成单位(BFU-E)数量受到抑制,在其叔叔中,加入血浆IgG组分后自体红系集落形成单位(CFU-E)数量受到抑制。在这些病例中,PRCA的发生被认为是由于血清抗体的存在基于自身免疫机制。这表明遗传易感性也可能是导致慢性获得性PRCA的一个重要因素。

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