title: Delayed hemolytic transfusion reaction in adult sickle-cell disease: presentations, outcomes, and treatments of 99 referral center episodes.
abstract: Delayed hemolytic transfusion reaction (DHTR) is one of the most feared complications of sickle-cell disease (SCD). We retrospectively analyzed the clinical and biological features, treatments and outcomes of 99 DHTRs occurring in 69 referral center patients over 12 years. The first clinical signs appeared a median of 9.4 [IQR, 3-22] days after the triggering transfusion (TT). The most frequent DHTR-related clinical manifestation was dark urine/hemoglobinuria (94%). Most patients (89%) had a painful vaso-occlusive crisis and 50% developed a secondary acute chest syndrome (ACS). The median [IQR] hemoglobin-concentration nadir was 5.5 [4.5-6.3] g/dL and LDH peak was 1335 [798-2086] IU/L. Overall mortality was 6%. None of the patients had been receiving chronic transfusions. Among these DHTRs, 61% were developed in previously immunized patients, 28% in patients with prior DHTR. Among Abs detected after the TT in 62% of the episodes, half are classically considered potentially harmful. No association could be established between clinical severity and immunohematological profile and/or the type and specificity of Abs detected after the TT. Management consisted of supportive care alone (53%) or with adjunctive measures (47%), including recombinant erythropoietin and sometimes rituximab and/or immunosuppressants. Additional transfusions were either ineffective or worsened hemolysis. In some cases, severe intravascular hemolysis can be likely responsible for the vascular reaction and high rates of ACS, pulmonary hypertension and (multi)organ failure. In conclusion, clinicians and patients must recognize early DHTR signs to avoid additional transfusions. For patients with a history of RBC immunization or DHTR, transfusion indications should be restricted. Am. J. Hematol. 91:989-994, 2016. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Unité des Maladies Génétiques du Globule Rouge, APHP, Hôpitaux Universitaire Henri-Mondor, Créteil, France.|UPEC, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de le Recherche Médicale (INSERM) U955, DHU A-TVB, Créteil, France.
authors.forename: Anoosha
authors.initials: A
authors.lastname: Habibi
authors.affiliation: Service de Réanimation Médicale, Créteil, APHP, Hôpitux Universitaire Henri-Mondor, DHU A-TVB, France.|Faculté de Médecine, Groupe de Recherche CARMAS, Université Paris-Est Créteil, Créteil, France.
authors.forename: Armand
authors.initials: A
authors.lastname: Mekontso-Dessap
authors.affiliation: Service de Médecine Interne, APHP, Hôpitaux Universitaire Henri-Mondor, Créteil, France.
authors.forename: Constance
authors.initials: C
authors.lastname: Guillaud
authors.affiliation: Service de Médecine Interne, APHP, Hôpitaux Universitaire Henri-Mondor, Créteil, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Michel
authors.affiliation: Service de Réanimation Médicale, Créteil, APHP, Hôpitux Universitaire Henri-Mondor, DHU A-TVB, France.
authors.forename: Keyvan
authors.initials: K
authors.lastname: Razazi
authors.affiliation: UPEC, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de le Recherche Médicale (INSERM) U955, DHU A-TVB, Créteil, France.|Service des Urgences, APHP, Hôpitaux Universitaire Henri-Mondor, Créteil, France.
authors.forename: Mehdi
authors.initials: M
authors.lastname: Khellaf
authors.affiliation: Faculté de Médecine Créteil, Établissement Français du Sang (EFS) Île-de-France, Créteil, France.
authors.forename: Btissam
authors.initials: B
authors.lastname: Chami
authors.affiliation: Unité des Maladies Génétiques du Globule Rouge, APHP, Hôpitaux Universitaire Henri-Mondor, Créteil, France.
authors.forename: Dora
authors.initials: D
authors.lastname: Bachir
authors.affiliation: Service d'Hémovigilance, APHP, Hôpitaux Universitaire Henri-Mondor, Créteil, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Rieux
authors.affiliation: Service d'Immunologie Clinique, APHP, Hôpitaux Universitaire Henri-Mondor, Créteil, France.
authors.forename: Giovanna
authors.initials: G
authors.lastname: Melica
authors.affiliation: Service des Urgences, APHP, Hôpitaux Universitaire Henri-Mondor, Créteil, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.affiliation: Unité des Maladies Génétiques du Globule Rouge, APHP, Hôpitaux Universitaire Henri-Mondor, Créteil, France.|UPEC, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de le Recherche Médicale (INSERM) U955, DHU A-TVB, Créteil, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Galacteros
authors.affiliation: Unité des Maladies Génétiques du Globule Rouge, APHP, Hôpitaux Universitaire Henri-Mondor, Créteil, France.|UPEC, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de le Recherche Médicale (INSERM) U955, DHU A-TVB, Créteil, France.
authors.forename: Pablo
authors.initials: P
authors.lastname: Bartolucci
authors.affiliation: UPEC, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de le Recherche Médicale (INSERM) U955, DHU A-TVB, Créteil, France. france.pirenne@efs.sante.fr.|Faculté de Médecine Créteil, Établissement Français du Sang (EFS) Île-de-France, Créteil, France. france.pirenne@efs.sante.fr.
authors.forename: France
authors.initials: F
authors.lastname: Pirenne
mesh_terms: D056586:Acute Chest Syndrome; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D001157:Arterial Occlusive Diseases; D001803:Blood Transfusion; D000075202:Contraindications; D019468:Disease Management; D005260:Female; D006456:Hemoglobinuria; D006461:Hemolysis; D006801:Humans; D007518:Isoantibodies / Q000097:blood; D008297:Male; D012017:Referral and Consultation; D012189:Retrospective Studies; D013997:Time Factors; D065227:Transfusion Reaction / Q000175:diagnosis* / Q000628:therapy; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007518:Isoantibodies