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青少年慢性粒细胞白血病和皮肤组织细胞增多症。见于冯·雷克林豪森病。

Juvenile chronic myelogenous leukemia and dermal histiocytosis. In Von Recklinghausen's disease.

作者信息

Bestak M, Miller D R, Mouradian J S

出版信息

Am J Dis Child. 1979 Aug;133(8):831-3. doi: 10.1001/archpedi.1979.02130080071014.

Abstract

A 4-year-old boy with multiple café-au-lait spots and a family history of neurofibromatosis had generalized edema, histiocytic rash characterized by benign histiocytic infiltration, hepatomegaly, and life-threatening infection. Six months later, progessive splenomegaly led to juvenile chronic myelocytic leukemia that eventually proved fata. The case represents an important association of diseases.

摘要

一名4岁男孩有多处咖啡牛奶斑且有神经纤维瘤病家族史,出现全身水肿、以良性组织细胞浸润为特征的组织细胞性皮疹、肝肿大以及危及生命的感染。6个月后,进行性脾肿大导致青少年慢性粒细胞白血病,最终证明是致命的。该病例代表了一种重要的疾病关联。

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