A Rare Variant of Aggressive T-Cell Large Granular Lymphocyte Leukemia Associated With Hepatic Fibrosis and Trisomy 8: A Case Report and Literature Review

Sanjay de Mel, Benjamin Wong, Leena Gole, Siok Bian Ng, Evelyn Koay, Christopher Ng Wai Siong, Ju Ee Seet, Aileen Wee, Wee Joo Chng, Lip Kun Tan

Abstract


Aggressive T-cell large granular lymphocytic leukemia (T-LGL) is a rare entity with a poor prognosis, the main clinical findings being splenomegaly, lymphadenopathy, thrombocytopenia and a CD3+/CD56+ phenotype. A 21-year-old lady presented with hepatosplenomegaly, lymphadenopathy, “B” symptoms and thrombocytopenia. She subsequently developed jaundice, ascites and deranged liver function. LGL was detected in the peripheral blood and marrow. They had an aberrant CD3+/CD4-/CD8dim/CD56+ phenotype. Karyotyping showed trisomy 8 which has not previously been reported in aggressive T-LGL. A liver biopsy showed hepatic fibrosis with a sinusoidal LGL infiltrate. Her ascitic fluid also showed involvement by T-LGL. She had a poor response to cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) chemotherapy and opted for palliation. We performed targeted sequencing to detect mutations in the STAT3, STAT5b and PTPRT genes which were absent, suggesting the presence of an alternative genetic driver in our patient. We report an unusual case of aggressive T-LGL with an aberrant immunophenotype, trisomy 8 and hepatic fibrosis with absence of mutations in the STAT pathway. Our case describes novel features of aggressive T-LGL and the need for further studies to elucidate the genetic basis of this rare disease.




J Hematol. 2015;4(3):214-218
doi: http://dx.doi.org/10.14740/jh223w


Keywords


Large granular lymphocyte; Leukemia; Hepatic perisinusoidal fibrosis; CD56; Trisomy 8

Full Text: HTML PDF
 

Browse  Journals  

     

Journal of Clinical Medicine Research

Journal of Endocrinology and Metabolism

Journal of Clinical Gynecology and Obstetrics

World Journal of Oncology

Gastroenterology Research

Journal of Hematology

Journal of Medical Cases

Journal of Current Surgery

Clinical Infection and Immunity

Cardiology Research

World Journal of Nephrology and Urology

Cellular and Molecular Medicine Research

Journal of Neurology Research

International Journal of Clinical Pediatrics

 

 

 

 

 

Journal of Hematology, bimonthly, ISSN 1927-1212 (print), 1927-1220 (online), published by Elmer Press Inc.                            
The content of this site is intended for health care professionals.
This is an open-access journal distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License, which permits unrestricted
non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Creative Commons Attribution license (Attribution-NonCommercial 4.0 International CC BY-NC 4.0)



This journal follows the International Committee of Medical Journal Editors (ICMJE) recommendations for manuscripts submitted to biomedical journals,
the Committee on Publication Ethics (COPE) guidelines, and the Principles of Transparency and Best Practice in Scholarly Publishing.

website: www.thejh.org    editorial contact: editor@thejh.org
Address: 9225 Leslie Street, Suite 201, Richmond Hill, Ontario, L4B 3H6, Canada
 

© Elmer Press Inc. All Rights Reserved.


Disclaimer: The views and opinions expressed in the published articles are those of the authors and do not necessarily reflect the views or opinions of the editors and Elmer Press Inc. This website is provided for medical research and informational purposes only and does not constitute any medical advice or professional services. The information provided in this journal should not be used for diagnosis and treatment, those seeking medical advice should always consult with a licensed physician.