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[全身性IgG-λ浆细胞瘤与浆细胞浸润导致的Cimino分流管狭窄]

[Generalized IgG-lambda plasmocytoma and stenosis of a Cimino shunt caused by plasma cell infiltration].

作者信息

Reuss-Borst M A, Jacki S, Horny H P, Waller H D

机构信息

Abteilung Innere Medizin II, Universität sowie Pathologisches Institut, Tübingen.

出版信息

Dtsch Med Wochenschr. 1994 Dec 9;119(49):1694-8. doi: 10.1055/s-2008-1058889.

Abstract

A 56-year-old apparently healthy man was on routine examination found to have a paraproteinaemia, classified as an IgG-lambda plasmocytoma in stage 1 on the basis of a raised IgG level (1950 mg/dl) and partly binuclear plasma cells in bone marrow. When 5 months later the IgG concentration had increased to 2980 mg/dl and the proportion of plasma cells in bone marrow to 15%, treatment was begun with melphalan (0.25 mg/kg) and prednisolone (2 mg/kg), both on 4 successive days every 6 weeks. The patient's general condition rapidly worsened after 6 months. The extent of osteolysis increased, necessitating radiotherapy of the vertebrae as well as a change in treatment to vincristine, cyclophosphamide, doxorubicin and prednisolone. At that time renal failure set in. But the prophylactically placed Cimino shunt had become infiltrated with plasma cells causing shunt stenosis and soft-tissue swelling. The infiltration was reduced by irradiation with 18 Gy. Numerous bluish skin discolorations now appeared and plasma cell infiltrates were shown in lung and pleura, as well as in the abdomen. The patient died 15 months after the diagnosis had been made.

摘要

一名56岁看似健康的男性在常规检查中被发现患有副蛋白血症,根据升高的IgG水平(1950毫克/分升)和骨髓中部分双核浆细胞,被分类为1期IgG-λ浆细胞瘤。5个月后,当IgG浓度升至2980毫克/分升且骨髓中浆细胞比例达到15%时,开始使用美法仑(0.25毫克/千克)和泼尼松龙(2毫克/千克)进行治疗,每6周连续4天给药。6个月后患者的一般状况迅速恶化。骨溶解范围扩大,需要对椎体进行放疗,同时治疗方案改为长春新碱、环磷酰胺、阿霉素和泼尼松龙。此时出现了肾衰竭。但预防性放置的西米诺分流管已被浆细胞浸润,导致分流管狭窄和软组织肿胀。通过18戈瑞的照射,浸润情况有所减轻。现在出现了许多蓝色皮肤变色,肺、胸膜以及腹部均显示有浆细胞浸润。患者在确诊15个月后死亡。

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