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Transfusionsmedizin 2016; 6(03): 123-125
DOI: 10.1055/s-0042-110153
Kasuistik
Georg Thieme Verlag KG Stuttgart · New York

Erfolgreiche und anhaltende Induktion einer Immuntoleranz bei einem Patienten mit PNH und IgA-Anaphylaxie

Successful Long-term Induction of Immune Tolerance in a Patient with Paroxysmal Nocturnal Hemoglobinuria and IgA Anaphylaxis
B. Mayer
Institut für Transfusionsmedizin, Charité – Universitätsmedizin Berlin, Berlin
,
A. Salama
Institut für Transfusionsmedizin, Charité – Universitätsmedizin Berlin, Berlin
› Author Affiliations
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Publication History

Publication Date:
26 August 2016 (online)

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Zusammenfassung

Die Versorgung von Patienten mit anaphylaktoiden Transfusionsreaktionen aufgrund von Antikörpern gegen IgA ist in der Regel schwierig, insbesondere, wenn notfallmäßig oder über einen längeren Zeitraum Blutprodukte verabreicht werden müssen. Wir berichten über einen Patienten mit aplastischer Anämie bei PNH-Überlappungssyndrom und schwerer, mehrfach aufgetretener IgA-Anaphylaxie. Nach erfolgreicher Induktion einer Immuntoleranz vor ca. 2,5 Jahren wird der Patient mit Standardpräparationen von Erythrozytenkonzentraten (EK) versorgt. Zur Aufrechterhaltung der Immuntoleranz werden im Abstand von 2–3 Wochen regelmäßig IgA-haltige Immunglobuline infundiert. Alle Transfusionen wurden ohne Komplikationen toleriert und führten zu einem adäquaten Anstieg des Hämoglobins. Eine Boosterung der Anti-IgA-Antikörper ist nicht erfolgt. Ferner stabilisierten sich die hämatologischen Parameter, sodass bisher auf eine ursprünglich geplante Transplantation allogener hämatopoetischer Stammzellen verzichtet werden konnte.

Abstract

Management of patients with IgA anaphylactic transfusion reactions due to IgA antibodies can be extremely difficult, especially if these patients need to be transfused in an emergency or require frequent administration of blood products. Here, we report on a patient with aplastic anemia in combination with PNH and a history of severe and recurrent episodes of IgA anaphylaxis. The patient has been transfused with standard RBCs following successful induction of immune tolerance 2.5 years ago. Maintenance of immune tolerance was achieved by regular infusion of IgA-containing immunoglobulines. All transfusions were tolerated without any side effects and resulted in an adequate increase of hemoglobin. There was no boosting of anti-IgA. Furthermore, hematological parameters improved, so initially planned allogeneic bone marrow transplantation has been abandoned.

Schlüsselwörter

IgA - Anaphylaxie - Transfusionsreaktion - Immuntoleranz - PNH

Key words

IgA - anaphylaxis - transfusion reactions - immune tolerance - PNH
 

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