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Abstract:
A syndrome has been described in which hypogammaglobulinaemia is associated with splenomegaly and haemolytic anaemia and also non-caseating granulomata in lymph nodes, liver, spleen and skin. This report describes a patient with this syndrome who in addition had a positive Kveim and elevated serum angiotensin converting enzyme (SACE), suggesting the diagnosis of sarcoidosis.