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Unusual Presentation of Aplastic Anaemia: Evolving into Myelodysplastic Syndrome with Excess Blasts 1 and Type III Paroxysmal Nocturnal Haemoglobinuria Clone

Authors :
Shilpi More
Saroj Rajput
Geetika Sharma
Nimisha Sharma
Tathagata Chatterjee
Source :
Journal of Clinical and Diagnostic Research, Vol 16, Iss 8, Pp ED10-ED12 (2022)
Publication Year :
2022
Publisher :
JCDR Research and Publications Private Limited, 2022.

Abstract

Aplastic Anaemia (AA) is an immune mediated, primary haematopoietic disorder characterised by pancytopenia with significant morbidity and mortality. Allogeneic Haematopoietic Stem Cell Transplant (HSCT) is the treatment of choice in younger patients whenever Human Leucocyte Antigen (HLA) matched donor is available. For older patients and those in whom matched donor is not available, immunosuppressive therapy is the frontline treatment. With the long survivals of AA patients, clonal evolution into Myelodysplastic Syndrome (MDS) and clinically evident Paraoxysmal Nocturnal Haemoglobinuria (PNH) is frequently seen over a period of 5-10 years. The prognosis and overall survival of post AA, MDS is poor and the only treatment of choice is Allogeneic HSCT. The overall survival of post AA, MDS is however comparable to de novo MDS post-transplant. The authors hereby discuss a case of 26-year-old male patient, known case of AA who evolved into MDS with Excess Blasts 1 (MDS-EB-1) and type III PNH clone over a period of six years.

Details

Language :
English
ISSN :
2249782X and 0973709X
Volume :
16
Issue :
8
Database :
Directory of Open Access Journals
Journal :
Journal of Clinical and Diagnostic Research
Publication Type :
Academic Journal
Accession number :
edsdoj.16ddf973c834af4823eae0a7ab04f4c
Document Type :
article
Full Text :
https://doi.org/10.7860/JCDR/2022/56765.16749