Background
Delayed haemolytic transfusion reaction (DHTR) is mainly caused by an immune response to transfused red blood cells (RBCs). Immunized patients have a high risk of producing antibodies in ...response to further transfusion. Controlling the immune response to RBCs is therefore a major goal in sickle cell disease (SCD).
Study design
We report an observational study of eight alloimmunized SCD patients with history of severe DHTR who were treated with rituximab before a new transfusion to prevent further immunization and DHTR.
Results
Five patients showed a good clinical outcome following transfusion preceded by preemptive treatment with rituximab. The remaining patients presented mild DHTR. In all patients, the results of post‐transfusion screening tests were identical to those of pretransfusion tests; no newly formed antibodies were detected.
Conclusion
These cases suggest that rituximab prevents at least occurrence of newly formed antibodies in high responders and minimizes the risk of severe DHTR. This study confirms that DHTR is complex in SCD and does not rely only on the classical antigens/antibodies conflict. Considering potentially serious adverse effect of rituximab, this treatment should be considered cautiously, and only when transfusion is absolutely necessary in patients with history of severe DHTR linked to immunization.
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BFBNIB, DOBA, FZAB, GIS, IJS, IZUM, KILJ, NLZOH, NUK, OILJ, PILJ, PNG, SAZU, SBCE, SBMB, UILJ, UKNU, UL, UM, UPUK
1 Etablissement Français du Sang, Ile de France, Hôpital Henri Mondor, Créteil;
2 Centre de Référence des Cytopénies Auto-Immunes, Hôpital Henri Mondor, Créteil;
3 Service des Maladies Génétiques du ...Globule Rouge, Hôpital Henri Mondor Créteil;
4 Service de Médecine Interne, Hôpital Henri Mondor, Créteil;
5 Laboratoire dImmunologie, Hôpital Henri Mondor, Créteil;
6 Université de Poitiers, EA 3806, CHU de Poitiers
Correspondence: France Noizat-Pirenne, MD, PhD, Etablissement Français du Sang dIle de France, 51 Avenue du Maréchal de Lattre de Tassigny, 94000, Créteil, France. Tel: 331 56 72 76 37; Fax: 331 56 72 76 01, E-mail: france.noizat-pirenne{at}efs.sante.fr
Delayed hemolytic transfusion reaction (DHTR), a life-threatening transfusion complication in sickle cell disease (SCD), is characterized by a marked hemoglobin drop with destruction of both transfused and autologous red blood cells (RBCs) and exacerbation of SCD symptoms. One mechanism of RBCs destruction is auto-antibody production secondary to transfusion. As rituximab specifically targets circulating B cells, we thought that it could be beneficial in preventing this immune-mediated transfusion complication. We report the case of a SCD patient who previously experienced DHTR with auto-antibodies and who needed a new transfusion. DHTR recurrence was successfully prevented by rituximab administration prior transfusion, supporting the safe use of rituximab to prevent DHTR in SCD patients as a second line approach when other measures failed.
Key words: Sickle cell disease, rituximab, transfusion, auto-antibodies.
Transfusion of platelet concentrates Bierling, P
Transfusion clinique et biologique : journal de la Société française de transfusion sanguine
16, Issue:
2
Journal Article
Peer reviewed
Although a number of research have been realized in the aim to rationalize the use of platelet concentrates, these blood products remain absolutely necessary for patients with therapeutic aplasia and ...for some surgical patients. We will discuss in this work: the main rules of platelet transfusion procedures, threshold values for platelet transfusion, platelet doses, place of curative and prophylactic strategies, refractoriness to platelet transfusion, HLA immunization.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Background Transfusion‐related acute lung injury (TRALI) is currently one of the most common causes of transfusion‐related major morbidity and death. Among the many TRALI mediators, leucocyte ...antibodies have been identified as important triggers of severe TRALI.
Study Design and Methods These recommendations were compiled by experts of the ISBT Working Party on Granulocyte Immunobiology, based on the results obtained in eight international granulocyte immunology workshops, their personal experiences and on published study results.
Results Leucocyte antibody screening has to include the detection of human leucocyte antigen (HLA) class I, class II and human neutrophil alloantigen antibodies using established and validated techniques. HLA class I antibody detection should be restricted to antibodies clinically relevant for TRALI. To avoid unnecessary workload, TRALI diagnosis should be assessed by consultation with the reporting clinician and thorough exclusion of transfusion‐associated circulatory overload/cardiac insufficiency. In patients diagnosed with TRALI having donors with detectable leucocyte antibodies, evidence of leucocyte incompatibility should be provided by either cross‐matching or typing of patient for cognate antigen.
Conclusion Leucocyte antibody screening for the immunological clarification of TRALI cases as well as for identification of potentially alloimmunized blood donors is feasible and can be performed in a reasonable and quality assured manner. This practice can contribute to the prevention of antibody‐mediated TRALI.
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Transfusion de concentrés plaquettaires Bierling, P.
Transfusion clinique et biologique (Paris),
5/2009, Volume:
16, Issue:
2
Journal Article, Conference Proceeding
Peer reviewed
Bien que de nombreux travaux aient été réalisés pour rationaliser la transfusion de concentrés plaquettaires, ces produits restent largement utilisés pour les malades présentant une affection ...oncohématologique et dans certaines situations chirurgicales. Ce travail décrit les principales règles de la transfusion de concentrés plaquettaires : seuil transfusionnel, dose optimale de plaquettes à administrer, place respectives des attitudes curative et prophylactique, conduite à tenir en présence d’une thrombopénie réfractaire et/ou de la survenue d’une allo-immunisation anti-HLA.
Although a number of research have been realized in the aim to rationalize the use of platelet concentrates, these blood products remain absolutely necessary for patients with therapeutic aplasia and for some surgical patients. We will discuss in this work: the main rules of platelet transfusion procedures, threshold values for platelet transfusion, platelet doses, place of curative and prophylactic strategies, refractoriness to platelet transfusion, HLA immunization.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
To evaluate the response to treatment in a large cohort of patients with systemic lupus erythematosus (SLE) associated with autoimmune thrombocytopenia.
Response to treatment was assessed ...retrospectively in 59 patients with SLE, either definite (n = 44) or incomplete (n = 15), associated with frank autoimmune thrombocytopenia (defined as platelet count < 50 x 10(9)/l). Response to treatment was classified as complete (CR: platelet count > 150 x 10(9)/l), partial (PR: platelet count > 50 x 10/l), or failure (FR) in the other cases.
Oral prednisone alone was used in 50 of the 59 patients (mean initial dose 1 mg/kg body weight/day). A response was obtained in 80% of cases (CR in 28. PR in 12) but only 11 (22%) had a sustained response (CR, n = 7; PR, n = 4). In contrast, combined treatment with prednisone and either danazol (n = 18) or hydroxychloroquine (n = 11) resulted in 50% (7 CR, 2 PR) and 64% (4 CR, 3 PR) longterm responses, respectively, allowing prednisone to be withdrawn or the dose tapered below 0.2 mg/kg body weight/day. High dose methylprednisolone pulses (n = 10) and intravenous immunoglobulin (IVIG) (n = 31) resulted in positive responses in 60% (4 CR, 2 PR) and 65% (12 CR, 8 PR) of cases, respectively, but the response was transient in each case. Splenectomy (n = 17) resulted in 65% longterm responses (10 CR, 1 PR). Only 2 longterm partial responses were obtained with the 22 immunosuppressant-containing regimens administered to 14 patients. At the end of the study. a response was observed in 52 (88%) patients CR: 36 (61%), PR: 16 (27%), mainly as a result of splenectomy or combined treatment with prednisone and either danazol or hydroxychloroquine.
Longterm remission was obtained in the majority of patients. The major treatments inducing remission were splenectomy and prednisone combined with danazol or hydroxychloroquine.
A retrospective analysis was conducted on 20 D− liver transplant (LT) recipients transfused with D+ RBCs perioperatively and screened for RBC antibodies between 2 and 6 months later. None developed ...anti‐D detectable by the indirect antiglobulin test. Two patients produced weak anti‐D that reacted only with papain‐treated RBCs at 10 and 11 days without any sign of immune haemolysis. Antibodies became quickly undetectable. These data suggest an unusual pattern of alloimmunization in LT recipients with rapid, weak and transient antibody response and support the safety of transfusing D+ RBCs in most of D− patients during LT surgery.
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BFBNIB, DOBA, FZAB, GIS, IJS, IZUM, KILJ, NLZOH, NUK, OILJ, PILJ, PNG, SAZU, SBCE, SBMB, UILJ, UKNU, UL, UM, UPUK
Since its creation in 1993, hemovigilance has an important place for blood safety. The part concerning donors, as the name suggests, targeted on improvement of donor's safety covers in fact the two ...points of the transfusion chain with serious adverse events in donor, epidemiologic survey for recipients and post-donation information on the two sides. Organized management and close collaboration between the actors of the transfusion chain are necessary to ensure the effectiveness of the system.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
9.
Thrombocytopenia and pregnancy Khellaf, M; Loustau, V; Bierling, P ...
La revue de medecine interne
33, Issue:
8
Journal Article
Peer reviewed
The occurrence of thrombocytopenia during pregnancy is frequent (about 10%). Etiologies of thrombocytopenia are dominated by the gestational thrombocytopenia (>75%), which requires no exploration and ...no specific treatment; it usually occurs during the last trimester of pregnancy and corrects itself spontaneously after delivery. Other etiologies are: (1) immune thrombocytopenia (ITP) either primary or associated with other pathologies; ITP may appear early in the first trimester of pregnancy, (2) thrombotic microangiopathy syndromes, and (3) obstetric thrombocytopenia: eclampsia and HELLP syndrome (hemolysis elevated liver enzymes, and low platelet count). Treatment of pre-eclampsia and HELLP syndrome is based on resuscitative measures and symptomatic fetal extraction that will be discussed according to the term and severity of the case. The treatment of microangiopathy is based on resuscitation and plasma exchange. For ITP, no specific action is needed during pregnancy and only symptomatic patients with a platelet count less than 30×10(9)/L must receive a treatment. It is important to prepare the childbirth that can be vaginally except if there is an obstetric contraindication. A platelet count of 50×10(9)/L is required for the delivery, and of 75×10(9)/L in case of spinal anesthesia. Treatment implies a short course of corticosteroids associated with infusion of immunoglobulins in the most severe forms or in case of steroids resistance. There is a risk of neonatal thrombocytopenia requiring a control of the blood count for the baby at birth and within 5 days, newborns have to be treated if the platelet count is less than 20×10(9)/L.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Depuis sa création en 1993, l’hémovigilance est au cœur de la sécurité transfusionnelle. Sa composante donneurs, bien que ciblée comme son nom l’indique sur l’amélioration de la sécurité du donneur, ...recouvre les deux extrémités de la chaîne transfusionnelle, avec les effets indésirables graves donneurs (EIGD) pour les donneurs, l’épidémiologie pour les receveurs de produits sanguins labiles (PSL) et les informations post-don (IPD) sur les deux versants. Une gestion organisée et une étroite collaboration entre les acteurs de la chaîne transfusionnelle sont essentielles pour assurer l’efficacité du système.
Since its creation in 1993, hemovigilance has an important place for blood safety. The part concerning donors, as the name suggests, targeted on improvement of donor's safety covers in fact the two points of the transfusion chain with serious adverse events in donor, epidemiologic survey for recipients and post-donation information on the two sides. Organized management and close collaboration between the actors of the transfusion chain are necessary to ensure the effectiveness of the system.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
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