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[呈现罕见表现的华氏巨球蛋白血症]

[Waldenström's macroglobulinemia with an uncommon presentation].

作者信息

Trueba F, Maslin J, Coutant G, Guymar S, Vigezzi J F, Desangles F, Nizou J-Y, Nicand E

机构信息

Laboratoire de biologie médicale, Hôpital du Val de Grâce, Paris.

出版信息

Ann Biol Clin (Paris). 2006 Jan-Feb;64(1):73-5.

Abstract

Waldenström's macroglobulinemia is a rare disease with an indolent clinical course. The median age of the affected patient is 65 years. Nevertheless, we report a case of Waldenström's macroglobulinemia revealed by a splenomegaly and severe pancytopenia, in a 51-year-old man without previous medical history. According to the recent consensus recommendations for the clinicopathological definition of Waldenström's macroglobulinemia, diagnosis was made through morphological and immunophenotypic data of medullary cells. The reduced survival of the patient is associated with the importance of the cytopenia.

摘要

华氏巨球蛋白血症是一种临床病程进展缓慢的罕见疾病。受影响患者的中位年龄为65岁。然而,我们报告了一例51岁、无既往病史的男性患者,其因脾肿大和严重全血细胞减少而被诊断为华氏巨球蛋白血症。根据最近关于华氏巨球蛋白血症临床病理定义的共识建议,通过骨髓细胞的形态学和免疫表型数据进行诊断。患者生存期缩短与血细胞减少的严重程度有关。

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