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Abstract : Rationale We report the morbidity of an agammaglobulinemic female in contrast to that of XLA. Methods This 13 y/o female has a history of recurrent sinopulmonary infections. Immunologic workup: panhypogammaglobulinemia; cytometry: <1% sIgM+, kappa/lambda+, CD19+, CD20+ PBL & BM lymphocytes. A CT scan demonstrated mild unilobar bronchiectasis. She developed respiratory distress with an O2 dependence. Repeat CT demonstrated diffuse (multilobar) bronchiectasis with cystic changes. Subsequently, she developed weight loss/enteritis. Endoscopy demonstrated duodenitis/villous atrophy resembling gluten-sensitive enteropathy. Recently, erythroid aplasia developed. Treatment consisted of: 1. High dose IVGG (1 gm/kg q 2 weeks) which resulted in an IgG trough of >800mg/dL, an 80% resolution of the diffuse bronchiectasis and cystic lesions, and a significant drop in O2 dependence. 2. Enteropathy clinically responded to a gluten and lactose free diet. 3. Erythroid aplasia transiently reversed after erythropoietin administration. In contrast, 2 brothers (17 & 19 years old) with XLA started IVGG replacement therapy at <2 years of age (500 mg/kg q 4 weeks; trough IgG of >750mg/dL). They have experienced no significant pulmonary, gastrointestinal or hematologic based morbidity. Results Although X-linked and autosomal agammaglobulinemia manifest a similar arrest in B lymphocyte differentiation at the pre-B or the pro-B cell stage, the clinical morbidity is more severe in some autosomal forms. This is exemplified by the current case. Conclusion Some forms of autosomal agammaglobulinemia may be associated with gene(s) that effect the function of B lymphocytes and other cell lineages and thus, result in a more severe immunodeficiency state.