[Advantages and difficulties of an erythrocytapheresis program for sickle-cell patients: a pediatric experience]

Transfusion programs are sometimes necessary to take care of severe sickle-cell patients. Treatment of cerebrovascular disease in sickle-cell disease is the most common indication. Periodic automated red blood cell exchange (erythrocytapheresis) is an alternative treatment. Sixteen patients less than 20 years old have been treated with chronic erythrocytapheresis since 2004 in the pediatric hematology and oncology department of the University Hospital of Rouen, 10 patients for cerebrovascular disease (1 was on secondary prevention and 9 were on primary prevention), 5 patients for pain crisis recurrence, and the last one for mild psychocognitive deficit disorder. This treatment was unsuccessful for 4 patients, 3 on primary prevention and 1 treated for pain crisis recurrence. These failures were caused by alloimmunization for 2 patients and venous access problems for 2 patients. For the other 12 patients, 5 of the 6 patients on primary prevention showed clear improvement (normalization of transcranial Doppler ultrasound or improvement on magnetic resonance angiography), the patient on secondary prevention had stability on cerebral MRI after 2 years of treatment, the 5 patients with pain crisis recurrence had good improvement, and psychocognitive abilities improved for the last patient. One hundred and ninety-nine erythroexchange sessions were performed for the 9 patients treated over a period of 10 to 30 months. Erythrocytapheresis sessions ran on average less than 1.5h. Three patients showed high ferritin levels at the beginning of erythroexchange, which normalized 2 to 10 months later. All patients reported better quality of life. Periodic erythroexchanges are an effective treatment for complicated sickle-cell anemia and iron overload. It requires human, material, and financial support, but not as much as simple transfusion or manual erythroexchange. Practical experience shows problems of venous access because of coagulation when sampling.