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Asian Pacific Journal of Nursing

Volume 10, Issue 2, 2023
Mcmed International
Asian Pacific Journal of Nursing
Issn
XXX-XXXX (Print), 2349 – 0683 (Online)
Frequency
bi-annual
Email
editorapjn@mcmed.us
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Abstract
Title
PAROXYMAL NOCTURNAL HEMOGLOBINURIA-A REVIEW
Author
Ranjana Verma* and Nancy Kurien
Email
ranjanaaiims@gmail.com
keyword
Monoclonal antibody, Thrombophilia, Haemoglobinuria.
Abstract
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare life-threatening and debilitating disorder of haematopoiesis. It is characterised by the clinical triad of Coombs-negative acquired extracorpuscular haemolytic anaemia, thrombophilia and bone marrow failure. The sequelae of intravascular haemolysis drastically affect the quality of life of patients. Cytopenia and in particular thrombophilia can cause life-threatening complications. Thromboembolic events are the major cause of deat. The only curative treatment for PNH is allogeneic stem cell transplantation from a related or unrelated stem cell donor. Recent reports demonstrated favourable outcomes. Other treatments were generally supportive in nature. Recently, eculizumab, a humanised monoclonal antibody that inhibits complement factor C5, was approved. It is a targeted, disease-modifying treatment of PNH.
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