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Long survival in sickle cell anemia
Author(s) -
Shurafa Muhammad S.,
Prasad Ananda S.,
Rucknagel Donald L.,
Kan Yuet Wai
Publication year - 1982
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830120407
Subject(s) - medicine , anemia , sickle cell anemia , aseptic necrosis , hemoglobin , fetal hemoglobin , hemolysis , pediatrics , gastroenterology , surgery , pregnancy , fetus , disease , biology , femoral head , genetics
Ten patients with sickle cell anemia surviving beyond the age of 40 were compared to 11 subjects with sickle cell anemia who died before that age. Hematologic and biochemical data as well as clinical and physical parameters of the two groups were compared. There was no statistically significant difference between the two groups with respect to the degree of anemia, severity of hemolysis, or hemoglobin A 2 levels. A statistically significant difference was found between the two groups with respect to fetal hemoglobin, red cell zinc, and secondary sexual characteristics. Although the long survivors had fewer crises per year than the short survivors, the long‐term complications such as leg ulcer, congestive heart failure, and aseptic necrosis of the hip were more common in the older patients. Cerebrovascular accidents were the cause of death in 9/11 short survivors and are absent in the long survivors. The α/β chain synthesis ratio was normal in the long‐survivor group and α gene mapping in five subjects in that group revealed the genotype αα/αα in four and — α/αα in one. The older patients as a group had higher red cell zinc values. The secondary sexual characteristics were also better developed in the older subjects. The overall significance of zinc status and of a higher HbF on longevity of sickle cell anemia patients remains unknown.