TY - CHAP M1 - Book, Section TI - Paroxysmal Nocturnal Hemoglobinuria (PNH) A1 - Lichtman, Marshall A. A1 - Kaushansky, Kenneth A1 - Kipps, Thomas J. A1 - Prchal, Josef T. A1 - Levi, Marcel M. PY - 2011 T2 - Williams Manual of Hematology, 8e AB - An acquired hematopoietic stem cell disorder characterized by deficiency of glycosyl phosphatidylinositol-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 leads to the complement-mediated intravascular hemolysis that is the clinical hallmark of the disease. Marrow failure and thrombophilia also complicate the disease.It is the only hemolytic anemia caused by an acquired intrinsic defect of the red cell. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/19 UR - hemonc.mhmedical.com/content.aspx?aid=1126652657 ER -