Familial haemophagocytic reticulosis. Report of 2 cases in sibs.
Two cases of familial haemophagocytic reticulosis occurring in sibs are described. The disease is characterised by a widespread proliferation of histocytes showing haemophagocytosis and it usually manifests clinically with haematological abnormalities. The initial presentation may be that of a meningo-encephalitic illness, the result of a lymphohistiocytic infiltration of the meninges and cerebral tissues. The clinical course and pathological features are described and the differential diagnosis is discussed.
D001344:Autopsy; D001853:Bone Marrow / Q000473:pathology; D001921:Brain / Q000473:pathology; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006644:Histiocytes; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis; D006801:Humans; D007223:Infant; D008198:Lymph Nodes / Q000473:pathology; D008206:Lymphatic Diseases / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D008297:Male; D008578:Meninges / Q000473:pathology; D010586:Phagocytes; D013154:Spleen / Q000473:pathology