戈谢病一例中脾脏的多个占位性病变。

Multiple space-occupying lesions of the spleen in a case of Gaucher's disease.

作者信息

Iwanami S, Namba K, Tsukada T, Hosaka T, Tagami M, Kubota A, Terada T

机构信息

Second Department of Internal Medicine, Sanraku Hospital, Tokyo, Japan.

出版信息

Intern Med. 1992 Apr;31(4):557-60. doi: 10.2169/internalmedicine.31.557.

DOI:10.2169/internalmedicine.31.557

PMID:1633369

Abstract

A 50-year-old female patient was admitted because of an enormously enlarged spleen and thrombocytopenia. Ultrasonography and magnetic resonance imaging revealed multiple space-occupying lesions in the spleen. She was diagnosed as having Gaucher's disease based on the low level of beta-glucosidase activity in leukocytes and Gaucher's cells present in bone marrow aspirate. Severe hypersplenism necessitated splenectomy. Pathological studies of the excised spleen, including ultrastructural examinations, demonstrated that multiple space-occupying lesions in the spleen were composed of typical Gaucher cells.

摘要

一名50岁女性患者因脾脏极度肿大和血小板减少入院。超声检查和磁共振成像显示脾脏有多个占位性病变。根据白细胞中β-葡萄糖苷酶活性水平低以及骨髓穿刺液中存在戈谢细胞，她被诊断为患有戈谢病。严重的脾功能亢进需要进行脾切除术。对切除脾脏的病理研究，包括超微结构检查，表明脾脏中的多个占位性病变由典型的戈谢细胞组成。