Primary `acquired' hypogammaglobulinaemia and amyloidosis

Primary "acquired" hypogammaglobulinaemia and amyloidosis.

The clinical details and post-mortem findings are given of a patient who had primary ;acquired' hypogammaglobulinaemia and was found at necropsy to have amyloidosis. The relationship of gamma globulin deficiency to amyloidosis is discussed, and the possible relevance of the hypogammaglobulinaemia to the formation and nature of amyloid is noted. It is suggested that a disordered immune response ...

Acquired hypogammaglobulinaemia and sarcoidosis.

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.

Primary hypogammaglobulinaemia and arthritis.

Arthritis may be the first clinical manifestation of primary hypogammaglobulinaemia. In 16 years of 281 patients who had immunodeficiency, 30 had arthritis at presentation. It was more common in Bruton's disease (15 (22%) of 69 patients) than in other forms of immunodeficiency (15 (7%) of 212 patients). Non-septic arthritis was more prevalent than septic arthritis, particularly monoarticular ar...

Acquired common variable hypogammaglobulinaemia and lymphoedema.

Two patients with lymphoedema of the legs developed recurrent respiratory infections and were found to have panhypogammaglobulinaemia. The early recognition and treatment of this form of immunity deficiency in patients with lymphoedema is important in order to prevent recurrent infections which could cause pulmonary damage and further lymphatic stasis.

Primary amyloidosis.