Limb amputation in hemoglobin SC disease after application of ice and elevation

A 31-year-old man with hemoglobin SC disease was admitted with acute chest syndrome, treated with antibiotics and ventilator support. His restrained right wrist and hand became swollen and cool, thought due to infiltration of an intravenous line. The hand was elevated, ice packs applied around the clock, and prophylactic low molecular weight heparin given. The arm quickly turned gangrenous. In spite of attempted thrombectomy and red blood cell exchange transfusion, amputation was required. Cold, elevation, and measures that reduce blood flow are known precipitators of sickling and crises. Vasoocclusion of the extremities to the point of gangrene is rarely reported in sickle cell disorders, and this case highlights the dangers of measures limiting blood flow in such patients.