○座談会

PNH診療の現状と今後の展望／司会：二宮治彦／出席者：川口辰哉／西村純一／Anita Hill／Richard Kelly／Wendell Rosse

○学会ハイライト

「第75回日本血液学会学術集会」印象記／七島　勉

・ASH 2013(American Society of Hematology)

　#1239 Preclinical Development and In Vivo Evaluation of Next-Generation Compstatin Analogs With Improved Systemic Profiles:A Novel Option For The Treatment Of Paroxysmal Nocturnal Hemoglobinuria

　#2466 Red Cell Complement Loading In PNH Patients On Eculizumab Is Associated With a C3 Polymorphism Which Influences C3 Function, Predicts For Increased Extravascular Hemolysis and Provides a Rationale For C3 Inhibition.

　#2477 Novel evidence that sphingosine-1-phosphate-mediated mobilization of hematopoietic stem/progenitor cells(HSPCs) during intravascular hemolysis requires attenuation of the SDF-1-CXCR4 retention axis of HSPCs in bone marrow niches - implications for paroxysmal nocturnal hemoglobinuria-induced mobilization of HSPCs.

　#594 Complement blockade with a C1 esterase inhibitor(human) in Paroxysmal nocturnal hemoglobinuria.

　#804 The presence of leukemogenic mutational events in paroxysmal nocturnal hemoglobinuria suggests that clonal architecture of bone marrow failure is similar to myelodysplastic syndrome.

○臨床ガイド

『難治性貧血の診療ガイド』

「PNHの診断基準と診療の参照ガイド」改訂の要旨／西村純一

○患者会紹介

「再生つばさの会」／インタビュー：小島勢二

「NPO法人 PNH倶楽部」／インタビュー：小峰光博

○Case Report

・重症感染症を併発し，エクリズマブの一時中断後，再投与を行ったPNHの一症例／得平道英

・再発性の急性腎不全および血栓症を併発するPNHに対するエクリズマブの使用経験／勝谷慎也　ほか

○新着文献紹介

・C5の遺伝子変異体とエクリズマブの奏効不良／コメント：小峰光博

　（N Engl J Med 370(7):632-639, 2014)

・PNH患者では補体受容体１遺伝子の遺伝子多型がエクリズマブの血液学的奏効と相関する／コメント：川口辰哉

　（Haematologica 99(2):262-266, 2014)