[Double heterozygotism SC. Clinical features].
Sickle cell hemoglobin C (SC) disease and homozygous sickle cell anemia (SS) share the same pathophysiology and most of their clinical manifestations. SC disease is marked by mild anemia, propensity to thrombotic complications and increased frequency of severe bacterial infections. These manifestations are usually less frequent and less severe than in the homozygous SS status. Nevertheless, two features require special comment: the proliferative retinopathy and the bone lesions (aseptic necrosis). These two "degenerative" complications are reported to be more frequent than in SS disease and may be related to the longivity of the SC patients. Although, SC disease generally runs a benign course, severe complications can occur at any age and a close follow-up is justified.
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Oksenhendler
D000755:Anemia, Sickle Cell / Q000097:blood / Q000235:genetics* / Q000503:physiopathology; D006580:Genetic Carrier Screening; D006444:Hemoglobin C / Q000032:analysis; D006451:Hemoglobin, Sickle / Q000032:analysis; D006720:Homozygote; D006801:Humans
D006451:Hemoglobin, Sickle; D006444:Hemoglobin C
Double hétérozygotisme SC. Aspects cliniques.