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Case report on spontaneous remission in paroxysmal nocturnal hemoglobinuria: a rare phenomenon.

Authors :
Hussain, Mussawair
Hussain, Fayyaz
Qamar, Unaiza
Khan, Safia
Ali, Syed Asif
Ahmed, Parvez
Source :
European Journal of Medical Case Reports; 2024, Vol. 8 Issue 1, p18-21, 4p
Publication Year :
2024

Abstract

Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder characterized by a deficiency of GPIanchored proteins in blood cells. It is associated with hemolysis, thromboembolic events, and bone marrow failure. Management includes complement inhibitors, anticoagulation, and stem cell transplantation. Spontaneous remission of PNH is rarely reported. Case Presentation: A 28-year-old male initially diagnosed with aplastic anemia developed portal vein thrombosis and was subsequently diagnosed with PNH. Due to the unavailability of complement inhibitors, anticoagulation therapy and supportive care were employed. Splenectomy with splenorenal shunt was performed for chronic thrombosis and associated complications. The patient achieved spontaneous remission with normalized blood counts and diminished PNH clone. Conclusion: Managing PNH in resource-limited settings presents challenges due to the unavailability of complement inhibitors. Spontaneous remission of PNH is rarely reported and needs further research. A multidisciplinary approach, accessibility to diagnostic tests, and advanced treatments will enhance PNH management in resource-limited settings. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
25204998
Volume :
8
Issue :
1
Database :
Complementary Index
Journal :
European Journal of Medical Case Reports
Publication Type :
Academic Journal
Accession number :
177135201
Full Text :
https://doi.org/10.24911/ejmcr.173-1696705928