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浆细胞白血病与高黏滞综合征。

Plasma cell leukemia and hyperviscosity syndrome.

作者信息

Geraci J M, Hansen R M, Kueck B D

机构信息

Department of Medicine, Medical College of Wisconsin, Milwaukee.

出版信息

South Med J. 1990 Jul;83(7):800-5. doi: 10.1097/00007611-199007000-00020.

Abstract

A 56-year-old man had dyspnea, weight loss, hemoptysis, and a generalized bleeding diathesis. Physical examination disclosed hepatosplenomegaly, congestive heart failure, and multiple sites of bleeding. Severe anemia, thrombocytopenia, rouleaux formation, and a leukocytosis with circulating immature plasma cells were observed, along with azotemia, hyperuricemia, and marked elevation of total proteins with a monoclonal IgG kappa spike. The finding of increased serum viscosity confirmed the clinical impression of the hyperviscosity syndrome. Emergency plasma exchange produced marked improvement in the clinical manifestations of hyperviscosity syndrome. Systemic chemotherapy resulted in a partial remission of the disease, but the patient ultimately died of complications of treatment. In this review, we discuss the diagnosis and management of the hyperviscosity syndrome.

摘要

一名56岁男性出现呼吸困难、体重减轻、咯血及全身出血倾向。体格检查发现肝脾肿大、充血性心力衰竭及多处出血。观察到严重贫血、血小板减少、缗钱状形成、白细胞增多伴循环中未成熟浆细胞,同时伴有氮质血症、高尿酸血症及总蛋白显著升高伴单克隆IgG κ峰。血清黏度增加的发现证实了高黏滞综合征的临床印象。紧急血浆置换使高黏滞综合征的临床表现有显著改善。全身化疗使疾病部分缓解,但患者最终死于治疗并发症。在本综述中,我们讨论高黏滞综合征的诊断和管理。

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