ACQUIRED HYPOGAMMAGLOBULINEMIA AND MULTI FOCAL GRANULOMATOSIS

In adult acquired hypogammaglobulinaemia multi focal granulomas have o ften been described and have regularly led to the hypothesis of an ass ociation with sarcoidosis. We present a case of this type in a man age d 29 who was a smoker with a hypoglobulinaemia involving IgG, IgA and IgM and which was discovered following pneumococcal pneumonias. He pre sented with a significant hepatosplenomegaly and absent cutaneous reac tions to T dependant antigens with an elevated ACE activity. Histologi cal examination of the splenectomy specimen and of the liver biopsy sh owed an infiltration by epithelioid follicles and confluent giant cell s without necrosis. The pulmonary studies showed a normal chest radiog raph but the bronchial biopsy again found a granulomatous infiltration . The broncho-alveolar lavage was cytologically normal and a very slig ht and paradoxical reduction of the alveolar immunoglobulins was noted implying either an active intraalveolar concentration of immunoglobul ins or a local synthesis. In the light of the few reported cases it se ems that the diagnosis of sarcoidosis should be dismissed here in favo ur of multi focal granulomatosis with hypogammaglobulinaemia. In hypog ammaglobulinaemia there is no clinical or biological method (IDR tuber culin, ACE, Kveim, histology) to confirm a superadded diagnosis of sar coidosis.