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Complete remission in advanced blastic NK‐cell lymphoma/leukemia in elderly patients using the hyper‐CVAD regimen
Author(s) -
Shapiro Michael,
Wasik Mariusz A.,
JunkinsHopkins Jacqueline M.,
Rook Alain H.,
Vittorio Carmela C.,
Itakura Haruka,
Frankel Matthew C.,
Georgala Sofia,
Schuster Stephen J.
Publication year - 2003
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.10381
Subject(s) - vincristine , medicine , cytarabine , terminal deoxynucleotidyl transferase , lymphoma , leukemia , regimen , cyclophosphamide , lymphoblastic lymphoma , oncology , cancer research , immunology , gastroenterology , chemotherapy , t cell , immunohistochemistry , immune system , tunel assay
Although its cell of origin is still controversial, the blastic NK‐cell leukemia/lymphoma clearly represents a distinct type of hematopoietic neoplasm that is particularly clinically aggressive when it occurs in elderly patients as a disseminated, multi‐organ disease. Consistently effective treatments have not been developed for this malignancy. The present report describes two elderly patients with widespread blastic NK‐cell leukemia/lymphoma involving the skin, bone marrow, peripheral blood, lymph nodes, and viscera. In both cases the malignant cells were CD56+, CD2+, and terminal deoxynucleotidyl transferase (TdT) positive with no detectable T‐cell receptor (TCR) γ chain gene rearrangement. The cells also exhibited a low CD45 expression and strong CD99 (mic‐2) expression, as seen in immature lymphoid malignancies. The above findings support the precursor NK‐cell, rather than mature NK‐ or non‐NK‐cell, origin of the malignant cells. It is noteworthy that the two patients achieved complete responses to treatment with hyper‐CVAD (fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) alternating with high‐dose methotrexate/cytarabine, a regimen currently utilized in acute lymphoblastic leukemia and high‐grade lymphoma. The complete remission (CR) was sustained for 24 months in one patient who received four cycles (eight courses) of the treatment. It lasted 9 months in the second patient, who received only two cycles (four courses). If similar results are obtained with future patients, a randomized study comparing the hyper‐CVAD regimen to other therapeutic strategies may be warranted. Am. J. Hematol. 74:46–51, 2003. © 2003 Wiley‐Liss, Inc.