Delayed hemolytic transfusion reaction in adult sickle‐cell disease: presentations,outcomes, and treatments of 99 referral center episodes |
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| Authors: | Anoosha Habibi Armand Mekontso‐Dessap Constance Guillaud Marc Michel Keyvan Razazi Mehdi Khellaf Btissam Chami Dora Bachir Claire Rieux Giovanna Melica Bertrand Godeau Frédéric Galacteros Pablo Bartolucci France Pirenne |
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| Affiliation: | 1. Unité des Maladies Génétiques du Globule Rouge, APHP, H?pitaux Universitaire Henri‐Mondor, Créteil, France;2. UPEC, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de le Recherche Médicale (INSERM) U955, Créteil, France;3. Service de Réanimation Médicale, Créteil, APHP, H?pitux Universitaire Henri‐Mondor, DHU A‐TVB, France;4. Faculté de Médecine, Groupe de Recherche CARMAS, Université Paris‐Est Créteil, Créteil, France;5. Service de Médecine Interne, APHP, H?pitaux Universitaire Henri‐Mondor, Créteil, France;6. Service des Urgences, APHP, H?pitaux Universitaire Henri‐Mondor, Créteil, France;7. Faculté de Médecine Créteil, établissement Fran?ais du Sang (EFS) ?le‐de‐France, Créteil, France;8. Service d'Hémovigilance, APHP, H?pitaux Universitaire Henri‐Mondor, Créteil, France;9. Service d'Immunologie Clinique, APHP, H?pitaux Universitaire Henri‐Mondor, Créteil, France |
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| 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. |
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