Congenital CD59 Deficiency - 30/05/15
, Hubert Schrezenmeier, MD a, bRésumé |
The severe clinical symptoms of inherited CD59 deficiency confirm the importance of CD59 as essential complement regulatory protein for protection of cells against complement attack, in particular protection of hematopoietic cells and human neuronal tissue. Targeted complement inhibition might become a treatment option as suggested by a case report. The easy diagnostic approach by flow cytometry and the advent of a new treatment option should increase the awareness of this rare differential diagnosis and lead to further studies on their pathophysiology.
Le texte complet de cet article est disponible en PDF.Keywords : CD59 deficiency, Glycosylphosphatidylinositol anchor, Paroxysmal nocturnal hemoglobinuria, Terminal complement system, Membrane attack complex inhibitory factor, Homologous restriction factor, Membrane inhibitor of reactive lysis
Plan
| Dr B. Höchsmann and Dr H. Schrezenmeier have received research funding and honraria by the pharmaceutic industry for PNH activities. |
Vol 29 - N° 3
P. 495-507 - juin 2015 Retour au numéro
Article précédent
- Paroxysmal Nocturnal Hemoglobinuria : A Complement-Mediated Hemolytic Anemia
- Amy E. DeZern, Robert A. Brodsky
- Ultralarge Von Willebrand Factor–Induced Platelet Clumping and Activation of the Alternative Complement Pathway in Thrombotic Thrombocytopenic Purpura and the Hemolytic-Uremic Syndromes
- Nancy Turner, Sarah Sartain, Joel Moake
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