Trusted Resources: Evidence & Education
Scientific literature and patient education texts
Delayed hemolytic transfusion reaction in adult sickle-cell disease: presentations, outcomes, and treatments of 99 referral center episodes
source: American Journal of Hematology
authors: Habibi A, Mekontso-Dessap A, Guillaud C, Michel M, Razazi K, Khellaf M, Chami B, Bachir D, Rieux C, Melica G, Godeau B, Galacteros F, Bartolucci P, Pirenne Fsummary/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.organization: APHP, Hôpitaux Universitaire Henri-Mondor, France; Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de le Recherche Médicale (INSERM), France; Hôpitux Universitaire Henri-Mondor, France; Université Paris-Est Créteil, France; Établissement Français du Sang (EFS) Île-de-France, France
read more full text
Tandua Washington, MDDr. Tandua Washington has been a practic...
Low forced expiratory volume is associated with earlier death in sickle cell anemiaPulmonary complications result in mortal...
Family Celebrates 7-Year-Old’s Sickle Cell Cure Thanks to Sister’s Life-Saving GiftOne 7-year-old who suffered his whole li...
Genice Nelson, DNP, APRN, ANP-BCDr. Genice Nelson is a Nurse Practitione...
Mortality among California’s sickle cell data collection cohort, 2005-2015Information about age of death, cause of...
What is a bone marrow stem cell transplant?http://www.sicklecelltransplantconsortiu...
2023 Celebrating a Second Chance at Life Survivorship Symposium – VirtualWelcome to 2023 Celebrating a Second Cha...
The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.