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南非黑人的瓦尔登斯特伦巨球蛋白血症

Waldenström's macroglobulinaemia in South African Negroes.

作者信息

Sacher R A, Jacobson R J, Derman D, Schulman G

出版信息

Trop Geogr Med. 1978 Jun;30(2):207-14.

PMID:103261
Abstract

Waldenström's or primary macroglobulinaemia has rarely been documented in Black Africans. Six South African Negro patients with Waldenströms macroglobulinaemia were assessed and followed during a 10-year period from 1966 to 1976. The hyperviscosity syndrome was the presenting feature in four patients and one patient each presented with the nephrotic syndrome and a lacrimal mass respectively. On initial evaluation of the six patients, the serum IgM varied from 1.3 to 4.8 g% and in two patients cryoglobulinaemia was found. The patients received supportive treatment, chemotherapy, and when necessary, plasmaphoresis. One patient was lost to follow-up, one patient died 7 years after the onset of his illness, but the remaining four patients are alive 18 months to 5 years after presenting with their disease. Although the disease occurred in a younger age group (mean age 43 years) than in Caucasians it did not differ in clinical features or response to treatment from other parts of the world.

摘要

非洲黑人中很少有关于瓦尔登斯特伦巨球蛋白血症或原发性巨球蛋白血症的记录。1966年至1976年的10年间,对6例患有瓦尔登斯特伦巨球蛋白血症的南非黑人患者进行了评估和随访。高黏滞综合征是4例患者的首发症状,另有1例患者分别表现为肾病综合征和泪腺肿块。对这6例患者进行初步评估时,血清IgM水平在1.3至4.8 g%之间,2例患者发现有冷球蛋白血症。患者接受了支持治疗、化疗,必要时还进行了血浆置换。1例患者失访,1例患者在发病7年后死亡,但其余4例患者在发病后18个月至5年仍存活。尽管该疾病在比白种人更年轻的年龄组(平均年龄43岁)中出现,但其临床特征或对治疗的反应与世界其他地区并无差异。

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