Thrombotische Mikroangiopathie nach Stammzelltransplantation

K. Kentouche

doi:10.1055/s-0037-1619600

Hämostaseologie, Table of Contents

Hamostaseologie 2004; 24(01): 77-83
DOI: 10.1055/s-0037-1619600

Grußwort

Schattauer GmbH

Thrombotische Mikroangiopathie nach Stammzelltransplantation

Thrombotic microangiopathy following stem cell transplantation

K. Kentouche

¹Abt. Hämatologie, Onkologie und Immunologie (Leiter: Prof. Dr. F. Zintl), Klinik für Kinder- und Jugendmedizin, Friedrich Schiller Universität Jena

› Author Affiliations

Abstract

Zusammenfassung

Die Stammzelltransplantation-assoziierte thrombotische Mikroangiopathie (TA-TMA) tritt bei ca. 7% aller Transplantationen auf. Während sie alle Zeichen der idiopathischen TTP oder des HUS aufweist, zeigt die Plasmatherapie kaum Erfolg. Insgesamt versterben mehr als 80% der TA-TMA-Patienten. Der Vergleich von Fallberichten konnte Risikofaktoren für die Entwicklung einer TA-TMA aufdecken und unterschiedliche Varianten der Erkrankung anhand klinischer und labordiagnostischer Parameter sowie der therapeutischen Beeinflussbarkeit definieren. Es wird eine multifaktorielle Ätiologie angenommen, deren zentrale Gemeinsamkeit die Endothelschädigung ist. Das Gerinnungssystem wird durch intensive chemo- und strahlentherapeutische Konditionierungsbehandlung, Infektionen und Graft-versus-host-Reaktion in Verbindung mit der immunsuppressiven Therapie (v. a. Cyclosporin A) in Richtung prokoagulanter Status verschoben. Die bisherigen von der idiopathischen TTP übernommenen Therapieansätze verliefen enttäuschend. Das Absetzen von Cyclosporin ist der einzige Konsens in diesem Zusammenhang. Zukünftige Behandlungsansätze zielen auf Minimierung des Endothelschadens.

Summary

Stem cell transplantation associated microangiopathy (TA-TMA) occurs in about 7% of all transplantations. While it resembles in all its features idiopathic TTP or HUS, response to plasma therapy is poor. The overall mortality of patients afflicted with TA-TMA is about 80%. Comparative studies aimed at discovering risk factors for the development of TA-TMA and defined different entities according to clinical, laboratory and outcome parameters. It is thought to be of multifactorial aetiology with the central event of endothelial damage. Intensive chemotherapy and radiation for bmt-preparation, infection in the immunocompromised host and graft versus host disease in conjunction with immunosuppressive therapy i. e. cyclosporine turn the haemostatic balance into a procoagulant state. Therapeutic strategies deduced from idiopathic TTP were disappointing with the only consensus in the withdrawal of cyclosporine from TA-TMA patients. Future trials are directed to ameliorate endothelial dysfunction.

Schlüsselwörter

Thrombotische Mikroangiopathie - Stammzelltransplantation - Endothelschaden

Keywords

Thrombotic microangiopathy - stem cell transplantation - endothelial dysfunction

Full Text

References

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