RT Book, Section
A1 Lichtman, Marshall A.
A1 Kaushansky, Kenneth
A1 Kipps, Thomas J.
A1 Prchal, Josef T.
A1 Levi, Marcel M.
SR Print(0)
ID 1126652657
T1 Paroxysmal Nocturnal Hemoglobinuria (PNH)
T2 Williams Manual of Hematology, 8e
YR 2011
FD 2011
PB McGraw-Hill Education
PP New York, NY
SN 9780071622424
LK hemonc.mhmedical.com/content.aspx?aid=1126652657
RD 2024/04/18
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.