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. Author manuscript; available in PMC: 2019 Apr 25.
Published in final edited form as: Semin Hematol. 2018 Apr 25;55(2):87–93. doi: 10.1053/j.seminhematol.2018.04.011

Table 3.

Commonly accepted indications for HCT in SCD

Each indication below has been associated with increased mortality, thus eligible for any types of HCT (with moderate to high risk of HCT related complications)
  • Stroke or clinically significant neurologic event or deficit lasting >24 hr

  • 2 or more episodes of ACS in the 2-year period preceding HCT, despite supportive care (ie. asthma therapy, HU, and/or regular transfusion program)

  • 2 or more VOC per year in the 2-year period preceding HCT, despite supportive care (ie, HU, pain management plan, and/or regular transfusion program)

  • tricuspid regurgitant jet velocity ≥2.7 m/s on echo

  • regular RBC transfusion therapy (≥8 transfusions per year for ≥ 1 yr) to prevent vaso-occlusive complications (ie, VOC, stroke, abnormal TCD, or ACS)

Each indication below has been associated with substantial morbidity, thus eligible for low risk HCT. If 2 or more indications, then eligible for moderate risk HCT in the context of clinical trials
  • Impaired neuropsychological function with abnormal cerebral MRI and angiography, Walters et al (2016)[38]

  • Sickle nephropathy (moderate or severe proteinuria, glomerular filtration rate 30 to 50% of the predicted normal value, or serum creatinine ≥1.5 times the upper limit of normal), Powars et al (1993)[45]

  • Sickle liver disease (direct bilirubin >0.4 mg/dL or ferritin >1000 ng/L), Feld et al (2015)[46]

  • Osteonecrosis of multiple joints

  • Red-cell alloimmunization during long-term transfusion therapy

Indications that have been largely replaced by the above criteria
  • Bilateral proliferative retinopathy with major visual impairment in at least one eye

  • Stage I or II sickle lung disease

HU, hydroxyurea; RBC, red blood cell; VOC, vaso-occlusive crisis; ACD, acute chest syndrome; TCD, transcranial Doppler testing