Allogeneic Hematopoietic Stem Cell Transplant

Allogeneic hematopoietic stem cell transplantation (HSCT) is the only potential cure for SCD. The best candidates are children with SCD who are younger than 16 years of age with severe complications, who have an identical HLA-matched donor, usually a sibling. The transplant related mortality rate is between 5% and 10% and graft rejection is approximately 10%. Other risks include secondary malignancies, development of seizures or intracranial bleeding, and infection in the immediate post-transplant period.5,34,35

Experience with HSCT in adult patients with SCD is very limited. Umbilical cord blood and hematopoietic cells from nonmatched donors are potential alternatives in some patients, but use is limited.5,35

Acute Complications

Transfusions for Acute Complications Red cell transfusion is indicated in patients with acute exacerbations of baseline anemia; in cases of severe vasoocclusive episodes, including ACS, stroke, and acute multiorgan failure; and in preparation for procedures that will require the use of general anesthesia or ionic contrast products. Transfusions also may be useful in patients with complicated obstetric problems, refractory leg ulcers, refractory and prolonged pain crises, or severe priapism. Hyperviscosity may occur if the hemoglobin level is increased to greater than 10 to 11 g/dL (100-110 g/L or 6.2-6.8 mmol/L). Volume overload leading to congestive heart failure is more likely to occur if the anemia is corrected too rapidly in patients with severe anemia, and should be avoided.5,9

Infection and Fever Any fever greater than 38.5°C (101.3°F) in a SCD patient should be immediately evaluated, and the patient should have a blood culture drawn and be started on antibiotics that provide empirical coverage for encapsulated organisms. 9

Patients who should be hospitalized include the following:

• Infants younger than 1 year of age

• Patients with a previous sepsis or bacteremia episode

• Patients with temperatures in excess of 40°C (104°F)

• Patients with WBC counts greater than 30 x 107mm3 (30 x 109/L) or less than 0.5 x 103/mm3 (0.5 x 109/L) and/or platelets less than 100 x 103/mm3 (100 x 10 9/L) with evidence of other acute complications

• Acutely ill-appearing individuals

Broad IV antibiotic coverage for the encapsulated organisms can include ceftriax-one or cefotaxime. For patients with true cephalosporin allergy, clindamycin may be used. If staphylococcal infection is suspected due to previous history or the patient appears acutely ill, vancomycin should be initiated. Macrolide antibiotics, such as eryth-romycin or azithromycin, may be initiated if mycoplasma pneumonia is suspected. While the patient is receiving broad-spectrum antibiotics, their regular use of penicillin for prophylaxis can be suspended. Fever should be controlled with acetaminophen or ibuprofen. Because of the risk of dehydration during infection with fever, increased fluid requirements may be needed.5,9

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