Haplo T-Cell Depleted Transplantation in High-Risk Sickle Cell Disease

Inclusion Criteria

• •Homozygous Hemoglobin S Disease, or Hemoglobin S Beta0/+ thalassemia
• •Patients must demonstrate one or more of the following Sickle Cell Disease Complications
• •1. Clinically significant neurologic event (stroke) or any neurologic deficit lasting \>24 hours that is accompanied by an infarct on cerebral MRI
• •2. Minimum of two episodes of acute chest syndrome.
• •3. Recurrent painful events (at least 3 in the 2 years prior to enrollment).
• •4. Abnormal TCD study requiring starting on chronic transfusion therapy.
• •5. At least one silent infarct lesion on a MRI scan of the head.
• •A familial haploidentical donor without homozygous sickle cell disease
• •Adequate organ function (renal, liver, cardiac and pulmonary function)
• •Karnofsky or Lansky (age appropriate) Performance Score ≥50%
• •Liver biopsy is optional to assess for iron overload in chronically transfused patients.