RBC transfusion in sickle cell anemia (HbSS): experience from the Jamaican Cohort Study.


BACKGROUND: RBC transfusion is widely advocated in the management of sickle cell anemia (SCA), but it carries potentially serious risks, especially in the setting of chronic transfusion. The Jamaican Sickle Cell Clinic is conservative in its use of transfusion, and this experience is presented to allow comparison with other centers in defining the role for transfusion in SCA. STUDY DESIGN AND METHODS: Reported here is a retrospective, descriptive study of all RBC transfusions given to 311 subjects with SCA who were followed in a cohort study from birth and are, at this writing, 16.3 to 24.7 years old. RESULTS: There were 520 transfusion episodes in 197 (63.3%) of the 311 subjects; 1 transfusion in 80 (41%) of those who received transfusion(s), 2 transfusions in 54 (27%), 3 transfusions in 21 (11%), 4 in 17 (9%), 5 in 9 (5%), and 6 or more in 16 (8%). Single transfusions were usual for acute anemia of aplastic crises or acute splenic sequestration, and multiple transfusions (up to 21 episodes) were usual in the prophylaxis of recurrent stroke. Indications were aplastic crisis (102), acute chest syndrome (90), acute splenic sequestration (75), stroke (62), septicemia (46), hypoplasia (40), hypersplenism (34), surgery (31), gastroenteritis (10), and miscellaneous (30). CONCLUSION: Despite conservative transfusion use, 70.6 percent of patients had received at least one transfusion by 20 years of age in the Jamaican Sickle Cell Clinic: