Eculizumab before and after allogeneic hematopoietic stem cell transplantation in a patient with paroxysmal nocturnal hemoglobinuria/Paroksismal noktürnal hemoglobinürili bir ...
Yazarlar (9)
Hakan Göker
Burak Uz
Yahya Büyükasik
Salih Aksu
Ibrahim Haznedaroglu
Nilgün Sayinalp
Fatma Tekin
Osman İlhami Özcebe
Makale Türü Açık Erişim Özgün Makale (Uluslararası alan indekslerindeki dergilerde yayınlanan tam makale)
Dergi Adı Turkish Journal of Haematology
Dergi Tarandığı Indeksler
Makale Dili İngilizce Basım Tarihi 09-2011
Cilt / Sayı / Sayfa 28 / 3 / 223–0 DOI
Makale Linki https://jag.journalagent.com/tjh/pdfs/TJH_28_3_223_227.pdf
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Özet
Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by the triad of intravascular hemolysis, venous thrombosis, and cytopenia. Treatment of PNH is generally supportive. Bone marrow transplantation is the only curative therapy for PNH, but is associated with significant morbidity and mortality. Herein, we present a patient with PNH that received eculizumab, a humanized monoclonal antibody that blocks activation of the terminal complement at C5, before and immediately following allogeneic peripheral stem cell transplantation. Prior to hematopoietic stem cell transplantation eculizumab treatment markedly reduced hemolysis and transfusion requirement; however, 1 d post transplantation a hemolytic episode occured, which was successfully stopped with eculizumab re-treatment. Afterwards the patient did not require additional transfusions. The results of this study indicate that early administration of eculizumab may be a safe and effective therapy for hemolytic episodes associated with allogeneic peripheral stem cell transplantation in patients with PNH.(Turk J Hematol 2011; 28: 223-7)
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BM Sürdürülebilir Kalkınma Amaçları
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