The impact of red cell storage age on transfused patients with sickle cell disease: protocol of a pilot randomized clinical trial to evaluate changes in inflammation and clinical transfusion efficacy.

Background: Despite fulfilling all requirements for donor blood units as defined by the FDA, a number of patients with sickle cell disease (SCD) are transfused with red blood cell (RBC) units that are near the end of their storage life, exposing them to the potentially adverse components of the red cell storage lesion. Due to their chronically inflamed state, patients with SCD may be particularly susceptible to these components. We present here a pilot study protocol for testing the impact of fresh vs older red cell units in chronically transfused adults with SCD.

Nocturnal hypoxaemia is common in adults with sickle cell anaemia.

The symptoms and sequelae of sickle cell anaemia (SCA) are caused by the polymerization of deoxygenated sickle haemoglobin, and people with SCA may be uniquely susceptible to adverse outcomes from hypoxia and haemoglobin desaturation. We examined by oximetry adults (aged 18-45 years) with SCA presenting symptoms indicative of polysomnography, at a single institution, irrespective of treatment, for nocturnal hypoxaemia. Clinical labs and blood for in vitro assessments were taken upon enrolment and after 8-12 weeks of oxygen therapy or observation.