Description:

Two patients with sickle cell anemia are reported who developed relative impotence after repeated episodes of priapism. They learned that excessive ethanol ingestion produced an erection and utilized this mechanism to satisfy sexual partners. Hospitalization and transfusion therapy were required for repeated episodes of priapism. Extensive interviews were required in order to learn the sequence of events. This synergistic relationship between ethanol and priapism should be sought in patients with repeated hospitalizations for this complication of sickle cell anemia because it is preventable with appropriate counseling.  A review of the electrocardiograms (ECG) of 108 patients with sickle cell anemia found only 3 with patterns consistent with myocardial infarction. Two of the 3 patients with ECG infarct patterns had postmortem examination confirmation of the infarction. These two patients had no significant coronary atherosclerosis nor did the other six autopsied patients in the present series. Literature reports of postmortem examinations on patients with sickle cell anemia confirm the scarcity of coronary atherosclerosis and myocardial infarction in these patients. Forty of the 108 ECGs showed signs of left ventricular hypertrophy and 20 others had nondiagnostic ST and T wave abnormalities. Nine showed first degree AV block and four right bundle branch blocks. 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 haemolysis, or haemoglobin A2 levels. A statistically significant difference was found between the two groups with respect to fetal haemoglobin, 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.

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Sophia
Journal Coordinator
Global Journal of Research and Review