Multiorgan failure after sickle cell vaso occlusive attack: integrated clinical and biological emergency

Cottin, Laurane; Rouvet, Camille; Homedan, Chadi; Conté, Mathieu; Mortaza, Satar; Rousselet, Marie‐Christine; Corby, Anne; Guyader, Maïlys Le; Zandecki, Marc; Reynier, Pascal

doi:10.1684/abc.2014.0996

Cited by 1 publication

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“…There are case reports and small series suggesting the efficacy of exchange transfusion in patients who are acutely unwell with life‐ or organ‐threatening complications, either primarily related to SCD (acute sickle hepatopathy) (Sheehy et al , ; Marti‐Carvajal & Marti‐Amarista, ) or unrelated (severe sepsis, multi‐organ failure) (Cottin et al , ). These severe illnesses are associated with many factors that promote HbS polymerization, including both general and regional acidosis and hypoxia, and in these situations, we may consider transfusion after discussion with other specialists, including hepatologists and intensivists.…”

Section: Blood Transfusions In the Management Of Acute Complicationsmentioning

confidence: 99%

How I manage red cell transfusions in patients with sickle cell disease

2018

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Sickle cell disease is one of the commonest serious inherited diseases in the world, and red cell transfusion is still one of the few effective treatments for acute and chronic complications. Transfusion corrects anaemia and dilutes out the number of red cells able to cause vaso-occlusion and vascular damage. Urgent red cell transfusions are used to correct acute anaemia, treat acute chest syndrome and patients with acute neurological symptoms. We use elective transfusions preoperatively for moderate risk surgery, and in some pregnant women. There is good evidence for the use of long-term regular transfusions in primary stroke prevention, with the aim of keeping the percentage of sickle haemoglobin below 30%. Long-term transfusions are also used in secondary stroke prevention, and the management of progressive organ damage, including renal impairment and pulmonary hypertension. Blood needs to be matched for ABO, RH and Kell, although alloantibodies may still develop and require more careful, extended cross-matching. Delayed haemolytic transfusion reactions are relatively common, difficult to diagnose and manage, and potentially fatal.

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Section: Blood Transfusions In the Management Of Acute Complicationsmentioning

confidence: 99%