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[Familial hemophagocytic reticulosis (Farquhar) (author's transl)].

作者信息

Bergholz M, Rahlf G, Doering K M

出版信息

Pathol Res Pract. 1978 Nov;163(3):267-80. doi: 10.1016/s0344-0338(78)80020-x.

Abstract

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.

摘要

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