Bergholz M, Rahlf G, Doering K M
Pathol Res Pract. 1978 Nov;163(3):267-80. doi: 10.1016/s0344-0338(78)80020-x.
An account is given of six cases of familial hemophagocytic reticulosis (familial lymphohistiocytosis). After a period of illness varying from 2 weeks to 7.5 months the infants studied died with pancytopenia, fever and hepatosplenomegaly. Histologic examination revealed three characteristic features: Lymphocyte infiltration of the organs, reduction of the lymphatic and hematopoetic system, histiocytosis with erythrophagocytosis. The etiology of this disease remains obscure. Congenital allogeneity may be a possibility, but further immunologic investigations would be necessary to support this hypothesis.
本文报道了6例家族性噬血细胞性网状细胞增生症（家族性淋巴细胞组织细胞增生症）。在经过2周至7.5个月不等的患病期后，所研究的婴儿均死于全血细胞减少、发热和肝脾肿大。组织学检查显示三个特征性表现：器官淋巴细胞浸润、淋巴和造血系统减少、伴有红细胞吞噬现象的组织细胞增多症。本病的病因仍不清楚。先天性异体反应性可能是一种原因，但需要进一步的免疫学研究来支持这一假说。
