Management of paroxysmal nocturnal hemoglobinuria: an update

DOI Web Site PubMed

Bibliographic Information

Other Title
  • 発作性夜間ヘモグロビン尿症（PNH）
  • 発作性夜間ヘモグロビン尿症(PNH) : 最新の治療指針
  • ホッサセイ ヤカン ヘモグロビン ニョウショウ(PNH) : サイシン ノ チリョウ シシン
  • —最新の治療指針—

Search this article

Description

<p>The accepted therapeutic strategy for treating paroxysmal nocturnal hemoglobinuria (PNH) has drastically changed since eculizumab was introduced in 2010 in Japan. Its clinical impact has paved way for the new area of anti-complement therapy, and it has sparked further research and development of anti-complement drugs. The long-term safety and efficacy of eculizumab was established in clinical settings by analyzing post-marketing surveillance that is mandatory in Japan and in clinical trial settings. However, new clinical complications such as resistance to eculizumab, breakthrough hemolysis, extravascular hemolysis, and invasive meningococcal disease (IMD) have emerged after the introduction of eculizumab. Therefore, it is important to strategize effective ways to tackle these issues occurring during eculizumab treatment. In Japan, it is an urgent priority to reach a consensus for managing IMD in patients treated with eculizumab and for establishing protocols for prophylaxis to prevent meningococcal infection.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 59 (6), 703-710, 2018

    The Japanese Society of Hematology

Keywords

Details 詳細情報について

Report a problem

Back to top