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IgA deficiency and autoimmune haemolytic anaemia.

Selective IgA deficiency is often connected with autoimmune diseases. However, autoimmune haemolytic anaemia has only rarely been demonstrated in IgA deficient patients. This paper describes a 70-year-old woman with Coombs' positive haemolytic anaemia and IgA deficiency. With single radial immunodiffusion no IgA was detectable in serum, saliva and jejunal secretion. IgA-containing plasma cells were not seen by indirect immunofluorescence on jejunal biopsy specimens. Saliva contained the secretory component. The IgE concentration in serum was at the lower border of the normal range. IgG was slightly raised whereas IgM was normal. The patient suffered from frequent loose stols and recurrent respiratory and urinary tract infections. Clinical examination revealed a malabsorption syndrome whereas the histology of a jejunal biopsy was normal. The indirect Coombs' test was positive, showing IgG, IgM and complement on the red cells. Antibody specificity of the eluted immunoglobulins could not be detected. Autoantibodies against other tissues were not found. The family history revealed an unusual accumulation of diseases in siblings and parents: rheumatoid arthritis, thyreotoxicosis, mental diseases and spina bifida congenita. The IgG, IgA and IgM serum concentrations in four siblings were normal. The karyotype of the patient's lymphocytes was normal.