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June 1981

Deficient Activity of the Alternative Pathway of Complement in β Thalassemia Major

Author Affiliations

From the Department of Pediatrics, National Jewish Hospital and Research Center/National Asthma Center and University of Colorado School of Medicine, Denver (Drs Corry, Peerless, and Johnston and Ms Guthrie), and The Hospital for Sick Children, London (Dr Marshall).

Am J Dis Child. 1981;135(6):529-531. doi:10.1001/archpedi.1981.02130300029011

• Patients with thalassemia major suffer frequent and serious infections, especially after splenectomy. To explore the basis for this susceptibility, we examined activity of the complement system in sera from 24 patients. All sera had normal or increased activity of the classic complement pathway. However, six of the 24 (three with and three without splenectomy) had abnormal alternative pathway function, and mean alternative pathway activity was significantly decreased in both splenectomized and nonsplenectomized patients. Mean concentrations of C3, factor B, properdin, and immunoglobulins were normal. Defective alternative pathway function, especially in conjunction with asplenia, could contribute to the propensity to infection that exists in thalassemia.

(Am J Dis Child 1981;135:529-531)

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