title: [Multiorgan failure after sickle cell vaso occlusive attack: integrated clinical and biological emergency].
abstract: We describe the case of a 30-year-old patient, suffering from composite S/β + sickle cell disease. He was hospitalized following a vaso-occlusive attack with acute bone pains. Despite an analgesic treatment and transfusion of three units of red blood cells, a non-regenerative anemia appeared within 24 hours. One day later an acute chest syndrome with atelectasis of the left lung and desaturation and multi-organ failure occurred and necessitated the patient's intubation and required him to be placed in an artificial coma. A bronchoalveolar lavage was performed, which eliminated pneumonia but proved, after staining with oil red O, many neutral fatty acid microvacuoles in more than 80% of macrophages, suggesting a pulmonary fat embolism. The hypothesis of a bone marrow necrosis causing a pulmonary fat embolism was discussed and confirmed the next day by the characteristic appearance of the bone marrow. A therapeutic protocol associating iteratively bleeding and red blood cells transfusion was administered on the second day with the objective of maintaining haemoglobin S at less than 20% rate. Successive haemoglobin S assay was applied using a high performance liquid chromatography (HPLC) technique with a quick response within one hour after transfusion or bleeding. This protocol resulted in an improvement in the patient's condition, with a gradual normalization of vital signs and extubation twelve days later and discharge without sequelae twenty-five days later. The succession of rare but serious sickle cell complications anaemia which occurred in this patient could be controlled by adapting the laboratory for the clinical emergency.
authors.affiliation: Centre hospitalier universitaire, Département de biochimie et génétique, Angers, France.
authors.forename: Laurane
authors.initials: L
authors.lastname: Cottin
authors.affiliation: Centre hospitalier universitaire, Département de biochimie et génétique, Angers, France.
authors.forename: Camille
authors.initials: C
authors.lastname: Rouvet
authors.affiliation: Centre hospitalier universitaire, Département de biochimie et génétique, Angers, France.
authors.forename: Chadi
authors.initials: C
authors.lastname: Homedan
authors.affiliation: Centre hospitalier universitaire, Département de réanimation médicale, Angers, France.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Conté
authors.affiliation: Centre hospitalier universitaire, Département de réanimation médicale, Angers, France.
authors.forename: Satar
authors.initials: S
authors.lastname: Mortaza
authors.affiliation: Centre hospitalier universitaire, Département pathologie cellulaire et tissulaire, Angers, France.
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Rousselet
authors.affiliation: Centre hospitalier universitaire, Service des maladies du sang, Angers, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Corby
authors.affiliation: Centre hospitalier universitaire, Département d'hématologie et immunologie, Angers, France.
authors.forename: Maïlys
authors.initials: M
authors.lastname: Le Guyader
authors.affiliation: Centre hospitalier universitaire, Département d'hématologie et immunologie, Angers, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Zandecki
authors.affiliation: Centre hospitalier universitaire, Département de biochimie et génétique, Angers, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Reynier
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D019411:Clinical Laboratory Techniques; D004630:Emergencies; D006801:Humans; D008297:Male; D009102:Multiple Organ Failure / Q000209:etiology*
keywords: acute thoracic syndrome; bone marrow necrosis; bronchoalveolar lavage; fat embolism; sickle cell disease; vaso-occlusive crisis