[极端低丙种球蛋白血症伴肝脾肾浸润的大颗粒淋巴细胞增多症]

[Extreme hypogammaglobulinemia disclosing large granular lymphocytes with hepatosplenorenal infiltration].

作者信息

Harlé J R, Quinsat D, Durand J M, Horchowski N, Nicolino M, Kaplanski S, Farnarier C, Fossat C, Mongin M, Weiller P J

机构信息

Clinique médicale B, hôpital de la Timone, Marseille.

出版信息

Rev Med Interne. 1989 Jan-Feb;10(1):25-30. doi: 10.1016/s0248-8663(89)80109-2.

DOI:10.1016/s0248-8663(89)80109-2

PMID:2717825

Abstract

We report a case of large granular lymphocytosis, or chronic "natural killer" lymphocytosis, a newly described entity. We were able to demonstrate the proliferative character of the disease by the finding of karyotype abnormalities. This case was remarkable for the pre-existence, for at least three years, of severe hypogammaglobulinaemia, for the very slow course of the proliferative process and for the progressive and tumoral infiltration of the spleen and liver, then kidney.

摘要

我们报告一例大颗粒淋巴细胞增多症，即慢性“自然杀伤”淋巴细胞增多症，这是一种新描述的病症。通过发现核型异常，我们得以证实该疾病的增殖特性。该病例的显著特点是，严重低丙种球蛋白血症至少已存在三年，增殖过程进展非常缓慢，且脾脏、肝脏，随后是肾脏出现进行性肿瘤浸润。