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.

Autor: Hassan, Masroor, Abdullah, Hafez Mohammad Ammar, Wahid, Abdul, Qamar, Muhammad Ashraf
Zdroj: BMJ Case Reports; 6/12/2018, Vol. 2018, p1-4, 4p, 7 Color Photographs
Abstrakt: 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 TdTnegative T-cell lymphoblastic lymphoma. The patient failed to respond to chemotherapy on his relapse and died. [ABSTRACT FROM AUTHOR]
Databáze: Complementary Index