The disappearance of germinal centers in chronic lymphadeno-hepato-splenomegaly syndrome in childhood: report of three cases.
Three cases of a syndrome featuring massive splenomegaly, gross generalized lymphadenopathy, and moderate hepatomegaly are reported. Spleen weights ranged from 800 to 2400 g. Gradual depletion of lymphoid germinal centers, and prominent infiltration of the splenic and lymph node cords with plasma cells, immunoblasts and actively dividing B cells were the most distinctive histological features. The liver in two cases showed portal infiltrates. A marked hypergammaglobulinemia, a decrease in blood cholesterol level and hematological abnormalities related to hypersplenism were observed. The condition begins early in life and runs a chronic course, of up to 25 years. There was a family history in only one instance. Since there was no generalized immunodeficiency nor local depletion of T cells or dendritic reticulum cells, a failure in the local regulation of the immune response and possible cytokine production is postulated. This condition underlines the pivotal role of the local organization of the germinal centers in cellular cooperation and in the carrying out and regulation of the immune response.
Nezelof
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Unité d'Immunologie et d'Hématologie Pédiatriques (INSERM U 132), Hôpital Necker Enfants Malades, Paris, France.
Maupas
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Griscelli
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D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D005500:Follow-Up Studies; D006529:Hepatomegaly / Q000473:pathology*; D006801:Humans; D007223:Infant; D008099:Liver / Q000276:immunology / Q000473:pathology; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology; D008199:Lymphadenitis / Q000473:pathology*; D008297:Male; D013154:Spleen / Q000473:pathology; D013163:Splenomegaly / Q000473:pathology*; D013577:Syndrome