Eculizumab before and after allogeneic hematopoietic stem cell transplantation in a patient with paroxysmal nocturnal hemoglobinuria
Yazarlar (9)
Prof. Dr. Hakan Göker Hacettepe Üniversitesi, Türkiye
Burak Uz Hacettepe Üniversitesi, Türkiye
Prof. Dr. Yahya Büyükaşik Hacettepe Üniversitesi, Türkiye
Prof. Dr. Salih Aksu Hacettepe Üniversitesi, Türkiye
Ibrahim Haznedaroǧlu
Hacettepe Üniversitesi, Türkiye
Prof. Dr. Nilgün Sayinalp Hacettepe Üniversitesi, Türkiye
Dr. Öğr. Üyesi Yasemin KARACAN Hacettepe Üniversitesi, Türkiye
Fatma Tekin Hacettepe Üniversitesi, Türkiye
Prof. Dr. Osman Ilhami Özcebe Hacettepe Üniversitesi, Türkiye
Makale Türü Açık Erişim Özgün Makale (SCOPUS dergilerinde yayınlanan tam makale)
Dergi Adı Turkish Journal of Hematology
Dergi ISSN 1300-7777 Wos Dergi Scopus Dergi
Makale Dili İngilizce Basım Tarihi 01-2011
Cilt / Sayı / Sayfa 28 / 3 / 223–227 DOI 10.5152/tjh.2011.58
UAK Araştırma Alanları
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.
Anahtar Kelimeler
Complement | Eculizumab | Hemolytic episode | Paroxysmal nocturnal hemoglobinuria