Therapeutic red blood cell exchange in a child with sickle cell anaemia using the Spectra Optia? apheresis system

摘要

An 11-year-old African boy was suffering from the homozygous form of SCA, diagnosed at the age of 8 months when he was hospitalised because of bronchopneumonia and haemolytic anaemia. During the following 5 years, pain crises were rare (once a year) and the most important aim of therapy was pain relief, hydration and prophylaxis or treatment of infections. In 2006, the frequency of sickle cell (SC) crises increased and transfusion therapy became necessary - first every other month, and then monthly with the side effect of iron overload. For this reason, treatment with hydroxyurea was started, which was followed by a significant increase of haemoglobin (Hb) F and improvement of the patient's well-being. In 2010 and 2011, the frequency of pain crises increased again and monthly transfusions were needed. The decision to include the patient into a RBCX-programme after the first occurrence of an acute chest syndrome was based on the degree of iron overload despite chelation therapy, failure of hydroxyurea treatment, repeated pain crises and the ability to establish appropriate intravenous access.