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January 1976

IgA Deficiency and Autoimmune Hemolytic Disease: Report of Familial Occurrence and Discussion of the Implications of Transfusion Therapy

Author Affiliations

From the Blood Bank and the Department of Medicine A, Hadassah University Hospital and Medical School, Jerusalem, Israel.

Arch Intern Med. 1976;136(1):93-94. doi:10.1001/archinte.1976.03630010073014

Selective IgA deficiency is a common immunodeficient state that occurs in healthy individuals with a frequency ranging from 1:7001 to 1:3,0402, depending on population selection and diagnostic criteria. Selective IgA deficiency is associated also with a variety of clinical disorders, including chronic respiratory,3-5 gastrointestinal,6-8 and autoimmune diseases.9-11 Familial studies of IgA-deficient persons often detect relatives with other immunoglobulin deficiencies.12 Occasionally, selective IgA deficiency occurs in families as an autosomal-recessive trait,13-15 a dominant trait,16,17 or a dominant trait with intermediate inheritance.5 We report a mother with autoimmune hemolytic disease (AHD) of 20 years' duration and selective IgA deficiency, whose only child also has selective IgA deficiency. To our knowledge, this is the first report of familial selective IgA deficiency associated with AHD.

PATIENT SUMMARY  A 56-year-old Ashkenazi woman was admitted to the Hadassah-Hebrew University Medical Center for management of chronic hemolytic anemia.

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