[Familial lymphohistiocytosis. Anatomopathological study of 2 cases detected neonatally].
Two brothers presented with hemophagocytic reticulosis with neonatal onset. Early clinical and biological symptoms included pallor, hepatosplenomegaly and anemia, thrombocytopenia. Evolution was lethal in both cases, at 5 months and 13 days of age, respectively. Diagnosis was confirmed in both by the pathologic findings: diffuse lymphohistiocytic cellular proliferation with hemophagocytosis and atrophy of the lymphoid tissue. The diagnostic difficulties of this disease in the neonatal period are emphasized.
D006801:Humans; D007231:Infant, Newborn; D008099:Liver / Q000473:pathology; D008206:Lymphatic Diseases / Q000151:congenital / Q000235:genetics* / Q000473:pathology; D008297:Male
Lymphohistiocytose familiale. Etude anatomopathologique de 2 observations à révélation néonatale.