TY - CHAP M1 - Book, Section TI - Paroxysmal Nocturnal Hemoglobinuria A1 - Lichtman, Marshall A. A1 - Kaushansky, Kenneth A1 - Prchal, Josef T. A1 - Levi, Marcel M. A1 - Burns, Linda J. A1 - Linch, David C. Y1 - 2022 N1 - T2 - Williams Manual of Hematology, 10e AB - Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem/progenitor cell (HSPC) disorder characterized by deficiency of glycosyl phosphatidylinositol (GPI)-anchored proteins (GPI-APs) on the surface of hematopoietic cells. Two complement regulatory proteins (CD55 and CD59) are GPI anchored, and deficiency of these two proteins on erythrocytes derived from the mutant HSPC leads to complement-mediated intravascular hemolysis that is the clinical hallmark of the disease. Marrow failure and thrombophilia also complicate the disease. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/07 UR - hemonc.mhmedical.com/content.aspx?aid=1189334473 ER -