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7. Serum Immunoglobulin Levels in Sickle Cell Disease and Thalassemia Major

Author

Evans, Hugh E. and Reindorf, Carl

Abstract

Sickle CELL ANEMIA is frequently complicated by severe infection such as pneumococcal meningitis,1 salmonella osteomyelitis,2 and pneumonia.3 An immunologic basis for this predisposition has not been established. Thurman4 suggested that sickle cell anemia patients responded poorly to typhoid and tularemia vaccines but did not accumulate adequate data for statistical analysis. In contrast no deficiency in the response of such patients to salmonella vaccine was noted in a more recent study.5 Abnormalities in serum immunoglobulin levels have been observed in several diseases complicated by recurrent infection. To determine if such abnormalities occurred in sickle cell anemia, serum immunoglobulin values were investigated in pediatric patients with SS or SA hemoglobin patterns and in adults with SS hemoglobin patterns. Elevations of serum immunoglobulin levels, perhaps due, in part, to infection, were observed in patients with sickle cell anemia. In order to further elucidate these changes, patients with another hemoglobinopathy thalassemia major, were added

Published
1968
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8. Lung Function in Sickle Cell Hemoglobinopathy Patients Compared With Healthy Subjects

Author

Young, Roscoe C., Rachal, Raylinda E., Reindorf, Carl A., Armstrong, Earl M., Polk, Octavius D., Hackney, Robert L., and Scott, Roland B.

Subjects
Adult, Rest, Original Communications, Exercise Test, Humans, Anemia, Sickle Cell, Lung
Abstract

Previous studies of lung function tests performed on patients with sickle cell disease have shown a restrictive ventilatory defect, usually a diffusion defect, and mild hypoxia at rest. The present study was undertaken to explain the pathophysiology of these changes and to extend these studies to include functional measurements not reported previously.Lung function studies were performed at rest and during treadmill walking on 66 patients with sickle cell anemia and on 16 healthy control subjects. Patients had restrictive ventilatory defects, decreased lung compliance, and uneven ventilation-perfusion ratios. These abnormalities caused an increased alveolar-arterial oxygen tension difference that caused hypoxemia. The diffusion defects were because of the sickle cell disease.Carboxyhemoglobin levels were increased in patients with sickle cell disease. This increase may be caused by a combination of factors, including increased cigarette smoking, hemolysis, and preferential survival of red blood cells that contain carbon monoxide and which do not sickle. During treadmill walking, the patients with sickle cell disease showed a decreased work tolerance caused by impaired oxygen delivery. The anaerobic threshold is reached sooner in patients with sickle cell disease and may also account for the limitations in work capacity of these patients.

Published
1988

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