[Familial hemophagocytic reticulosis (Farquhar) (author's transl)].
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.
D001853:Bone Marrow / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D004912:Erythrocytes; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000473:pathology; D008206:Lymphatic Diseases / Q000235:genetics* / Q000473:pathology; D008297:Male; D010198:Pancytopenia / Q000209:etiology; D010587:Phagocytosis; D013154:Spleen / Q000473:pathology
Familiäre hämophagozytierende Retikulose (Farquhar).