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.