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Unusual presentation of more common disease/injury
CASE REPORT
Terminal deoxynucleotidyl transferase (TdT)-negative T-cell lymphoblastic lymphoma with loss of the T-cell lineage-specific marker CD3 at relapse: a rare entity with an aggressive outcome
  1. Masroor Hassan1,
  2. Hafez Mohammad Ammar Abdullah2,
  3. Abdul Wahid3,
  4. Muhammad Ashraf Qamar1
  1. 1 Department of Pathology, Rehman Medical Institute, Peshawar, Pakistan
  2. 2 Department of Internal Medicine, Sanford School of Medicine, University of South Dakota, Sioux Falls, South Dakota, USA
  3. 3 Department of Oncology, Rehman Medical Institute, Peshawar, Pakistan
  1. Correspondence to Dr Hafez Mohammad Ammar Abdullah, ammar.abdullah{at}usd.edu

Summary

Terminal deoxynucleotidyl transferase (TdT)-negative T-cell lymphoblastic lymphoma is a variant of T-cell lymphoblastic lymphoma/T-cell lymphoblastic leukaemia. TdT is a marker of immaturity expressed in 90%–95% cases of lymphoblastic lymphoma and useful in differentiating it from other mature lymphomas/leukaemias. It has been associated with poorer response to chemotherapy and a more aggressive outcome. Here we present a case of TdT-negative T-cell lymphoblastic lymphoma in a 28-year-old man who presented with superior vena cava syndrome. The patient was treated with hyper-cyclophosphamide,vincristine, Adriamycin, dexamethasone (CVAD), however unfortunately suffered a relapse 1 year later. A unique feature of our case was that on relapse, the patient lost expression of the T-cell lineage-specific marker CD3, which has previously not been reported in association with TdT-negative T-cell lymphoblastic lymphoma. The patient failed to respond to chemotherapy on his relapse and died.

  • cancer intervention
  • haematology (incl blood transfusion)
  • oncology

https://doi.org/10.1136/bcr-2018-224570

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    Footnotes

    • Contributors MH and HMAA were responsible for writing the background and discussion. AW and MAQ were responsible for writing the case presentation and summary.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.