2014Journal of HematologyRequires access

Spontaneous Remission of Paroxysmal Nocturnal Hemoglobinuria During Eculizumab Treatment

Fahri Şahin, Asu Fergün Yılmaz, Melda Cömert, Füsun Özdemirkıran, Nihal Mete, Güray Saydam

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Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare acquired disease which is characterized by increased sensitivity to complement proteins. The only curative treatment option for PNH has been still allogeneic stem cell transplantation from HLA-matched donor. Eculizumab, which is a monoclonal antibody, has been established as the effective approach not only for prevention of hemolysis, but also for prevention of potential organ damages such as thromboses and failure of target organs. But eculizumab does not have any impact on, or may increase, clone status and nature of the disease. We, hereby, report a case whose disease undergone spontaneous remission under treatment of eculizumab treatment.

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What this paper is about

Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare acquired disease which is characterized by increased sensitivity to complement proteins. The only curative treatment option for PNH has been still allogeneic stem cell transplantation from HLA-matched donor. Eculizumab, which is a monoclonal antibody, has been established as the effective approach not only for prevention of hemolysis, but also for prevention of potential organ damages such as thromboses and failure of target organs. But eculizumab does not have any impact on, or may increase, clone status and nature of the disease. We, hereby, report a case whose disease undergone spontaneous remission under treatment of eculizumab treatment.

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Available abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare acquired disease which is characterized by increased sensitivity to complement proteins. The only curative treatment option for PNH has been still allogeneic stem cell transplantation from HLA-matched donor. Eculizumab, which is a monoclonal antibody, has been established as the effective approach not only for prevention of hemolysis, but also for prevention of potential organ damages such as thromboses and failure of target organs. But eculizumab does not have any impact on, or may increase, clone status and nature of the disease. We, hereby, report a case whose disease undergone spontaneous remission under treatment of eculizumab treatment.

Key concepts: Eculizumab, Paroxysmal nocturnal hemoglobinuria, Medicine, Hemoglobinuria, Immunology, clone (Java method), Monoclonal, Disease

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