Mortal quintet of sickle cell diseases

Sickle cell diseases (SCDs) are chronic inflammatory processes on capillary level. We tried to understand some possible correlations between stroke and severity of SCDs.

All patients with SCDs were taken into the study.

The study included 343 patients (174 males and 169 females). There were 30 cases (8.7%) with stroke. The mean ages were similar in both groups (32.5 versus 29.1 years in the stroke group and other, respectively, P>0.05). The female ratios were similar in both groups, too (43.3% versus 49.8%, respectively, P>0.05). Prevalences of associated thalassemia minors were also similar in them (73.3% versus 65.1%, respectively, P>0.05). Smoking was higher among the stroke cases, significantly (26.6% versus 13.0%, P<0.05). Mean white blood cell count, hematocrit value, and mean platelet count of the peripheric blood were similar in both groups (P>0.05 for all). On the other hand, although the painful crises per year, tonsilectomy, priapism, ileus, pulmonary hypertension, chronic obstructive pulmonary disease, coronary heart disease, chronic renal disease, rheumatic heart disease, avascular necrosis of bones, cirrhosis, and mortality were all higher in the stroke group, the differences were only significant for acute chest syndrome (ACS), digital clubbing, and leg ulcers (P<0.05 for all), probably due to the small sample size of the stroke group.

SCDs and smoking are chronic destructive processes on endothelium, and both terminate with early organ failures in life. Probably smoking, digital clubbing, leg ulcers, ACS, and stroke are mortal quintet of the SCDs that may indicate shortened survival in such patients.