Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease

Abstract

Background Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation. This is an update of a Cochrane Review first published in 2002, and last updated in 2013. Objectives To assess risks and benefits of chronic blood transfusion regimens in people with sickle cell disease for primary and secondary stroke prevention (excluding silent cerebral infarcts). Search methods We searched for relevant trials in the Cochrane Library, MEDLINE (from 1946), Embase (from 1974), the Transfusion Evidence Library (from 1980), and ongoing trial databases; all searches current to 04 April 2016. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register: 25 April 2016. Selection criteria Randomised controlled trials comparing red blood cell transfusions as prophylaxis for stroke in people with sickle cell disease to alternative or standard treatment. There were no restrictions by outcomes examined, language or publication status. Data collection and analysis Two authors independently assessed trial eligibility and the risk of bias and extracted data. Main results We included five trials (660 participants) published between 1998 and 2016. Four of these trials were terminated early. The vast majority of participants had the haemoglobin (Hb)SS form of sickle cell disease. Three trials compared regular red cell transfusions to standard care in primary prevention of stroke: two in children with no previous long-term transfusions; and one in children and adolescents on long-term transfusion. Two trials compared the drug hydroxyurea (hydroxycarbamide) and phlebotomy to long-term transfusions and iron chelation therapy: one in primary prevention (children); and one in secondary prevention (children and adolescents). The quality of the evidence was very low to moderate across different outcomes according to GRADE methodology. This was due to the trials being at a high risk of bias due to lack of blinding, indirectness and imprecise outcome estimates. Red cell transfusions versus standard care Children with no previous long-term transfusions Long-term transfusions probably reduce the incidence of clinical stroke in children with a higher risk of stroke (abnormal transcranial doppler velocities or previous history of silent cerebral infarct), risk ratio 0.12 (95% confidence interval 0.03 to 0.49) (two trials, 326 participants), moderate quality evidence. Long-term transfusions may: reduce the incidence of other sickle cell disease-related complications (acute chest syndrome, risk ratio 0.24 (95% confidence interval 0.12 to 0.48)) (two trials, 326 participants); incr...

Dépistage de la vasculopathie cérébrale drépanocytaire par doppler transcrânien au Mali

“…En cas de vitesse accélérée limite objectivée dans au moins une des artères, le risque d'AVC est de 2 % à 5 % par an, la surveillance par DTC doit être trimestrielle. Lorsqu'enfin, une vitesse accélérée pathologique est objectivée dans au moins une des artères, le risque d'AVC est de 10 % par an, un programme d'échange transfusionnel mensuel au long cours est recommandé car il a été clairement démontré que cette attitude réduit ce risque de manière significative [15,16]. La prévalence de DTC pathologiques chez les drépanocytaires SS et S/b 0 est comprise entre 10 et 15 % et celle des DTC limites, entre 9 et 17 % selon les études [17].…”

Blood Cell Membrane Omega-3 ( n -3) Fatty Acid Abnormality and Supplementation in Patients with Sickle Cell Anemia

“…Unfortunately, it is associated with a high rate of complications-transmission of infective agents, alloimmunization and transfusion reactions, and severe iron overload (Wang and Dwan, 2013). Hydroxyurea (HU), which is the only FDA approved pharmacological agent, is efficacious in reducing vasoocclusive crisis, blood transfusion requirement, ACS, organ damage and mortality in children and adults.…”

Intravenous infusion of haptoglobin for the prevention of adverse clinical outcome in Sickle Cell Disease

“…Currently, the guidelines surrounding this decision is determined by the specific disorder under consideration, for e.g. prevention via repeated blood transfusion is employed for ischemic stroke, whereas treatment with intensive wound care is employed for chronic leg ulcers [39,102]. The uncertainty of the natural history of renal SCD makes this a difficult question since the evidence is insufficient to determine if persons with micro-albuminuria will definitely progress to macro-albuminuria and onto renal failure and hence if 'prevention' would be due to the drug therapy or to non-progression.…”