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letter
. 1978 Jul;70(7):503–506.

Alternate Pathway Activation in Sickle Cell Disease and β-Thalassemia Major

Alfred C deCiutiis, Charles M Peterson, Margaret J Polley, Linda J Metakis
PMCID: PMC2537184  PMID: 702579

Abstract

Total hemolytic complement activity (CH50), immuno-electrophoretic conversion of Factor B (C3PA), and of C3 were studied in 16 patients with sickle cell disease in a steady state, eight patients in crisis, and ten patients with β-thalassemia major anemia maintained on a constant transfusion regimen. Patients with sickle cell disease in a steady state have moderatley 56 (percent) depressed conversion of Factor B in addition to markedly decreased conversion of C3 in four of ten patients. One of the three sickle cell patients and two of the four thalassemia patients with low C3 conversion levels have died subsequent to the studies. The combination of chronically decreased Factor B conversion in the face of markedly decreased C3 conversion may make these patients occasionally vulnerable to overwhelming infection analagous to the situation seen in postsplenectomy cases.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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