Sickle-cell-disease variants and pregnancy. A pathophysiologic report.

THE clinical symptoms of sickle-cell anemia result from a pathophysiologic involvement of the erythron, with severe anemia, occlusion of small blood vessels and hyperactivity of the bone marrow as manifestations. In the variants of sickle-cell disease symptoms may be minimal or totally absent, but occasionally, as a result of certain provocations, acute episodes can occur that rival in severity the "crisis" of sickle-cell anemia. Hemoglobin S thalassemia and hemoglobin S-C disease are considered the more pernicious of the variants of sickle-cell disease. When symptoms are present, the bone, spleen, lung and retina are the usual suspected sites of vascular occlusive . . .