Familial erythrophagocytic lymphohistocytosis. Report of two cases and clinicopathologic review.
An unusual syndrome of hepatosplenomegaly and fever followed by rapid deterioration and death has been described in 38 children from 21 families. Pancytopenia, liver dysfunction, and bleeding developed prior to death from hemorrhage, sepsis, or lymphocytic meningitis. This report reviews the literature and adds a set of twins to the reported cases.
D000917:Antibody Formation; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005334:Fever; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008206:Lymphatic Diseases / Q000097:blood / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D008297:Male; D011247:Pregnancy; D013154:Spleen / Q000473:pathology