Table 2.

Clinical vignettes.

Case  1: A 16-year-old boy with sickle cell anemia (Hb SS) is scheduled for elective laparoscopic cholecystectomy. The baseline labs reveal that Hct is 22% and Hb is 7.2.
Case  2: A 4-year-old girl with known sickle cell anemia (Hb SS) presents to the Emergency Department with a 12-hour history of abdominal pain, nausea, vomiting, and lethargy. Physical examination reveals an easily palpable and tender spleen. The CBC shows WBC 29,000/μL with 80% neutrophils and 12% bands, and Hct 12%, and platelets 88,000/μL. The physician in charge requests assistance in transfusion recommendations.
Case  3: A 28-year-old woman with Hb SC disease has acute chest syndrome with progressive hypoxemia, despite oxygen supplementation. Review of the CBC reveals WBC 22,000/μL, Hct 28%, and platelets 530,000/μL.
Case  4: A 21-year-old male with sickle cell anemia (Hb SS) would like to enter your practice. The patient has been undergoing transfusions of 2 units packed RBCs, every 4 weeks since a stroke at age 12 with a goal to maintain his Hb S level at ~50%. He has been on deferoxamine therapy over the past 7 years.