Type:

Other

Description:

Physiology in Medicine review article. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem-cell disorder caused by a somatic mutation in a gene known as phosphatidylinositol glycan class A (PIGA). It may arise de novo or in the setting of acquired aplastic anemia.The absence of GPI-anchored proteins leads to complement-mediated intravascular hemolysis, because 2 important complement regulatory proteins (CD55 and CD59) are missing from PNH cells. Therapeutic options include supportive care, bone marrow transplantation, and monoclonal antibody therapy with the terminal complement inhibitor eculizumab.

Subjects:

  • Education > General

Education Levels:

    Keywords:

    oai:nsdl.org:2200/20110722024315740T,NSDL,NSDL_SetSpec_BEN,Stem cell,Life Science,Teacher-centered/traditional instruction,Education,Anemia

    Language:

    English

    Access Privileges:

    Public - Available to anyone

    License Deed:

    Creative Commons Attribution Non-Commercial Share Alike

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