title: Complete remission in advanced blastic NK-cell lymphoma/leukemia in elderly patients using the hyper-CVAD regimen.
abstract: 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) gamma 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.
authors.affiliation: Department of Dermatology, Division of Hematology-Oncology, University of Pennsylvania Health System, Philadelphia, Pennsylvania 19104, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Shapiro
authors.forename: Mariusz A
authors.initials: MA
authors.lastname: Wasik
authors.forename: Jacqueline M
authors.initials: JM
authors.lastname: Junkins-Hopkins
authors.forename: Alain H
authors.initials: AH
authors.lastname: Rook
authors.forename: Carmela C
authors.initials: CC
authors.lastname: Vittorio
authors.forename: Haruka
authors.initials: H
authors.lastname: Itakura
authors.forename: Matthew C
authors.initials: MC
authors.lastname: Frankel
authors.forename: Sofia
authors.initials: S
authors.lastname: Georgala
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Schuster
mesh_terms: D000368:Aged; D000964:Antimetabolites, Antineoplastic / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000627:therapeutic use*; D003561:Cytarabine / Q000008:administration & dosage; D003907:Dexamethasone / Q000627:therapeutic use*; D004317:Doxorubicin / Q000627:therapeutic use*; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D007938:Leukemia / Q000188:drug therapy* / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000188:drug therapy* / Q000473:pathology*; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D008875:Middle Aged; D012074:Remission Induction; D014750:Vincristine / Q000627:therapeutic use*
chemical_list: D000964:Antimetabolites, Antineoplastic; D003561:Cytarabine; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D003520:Cyclophosphamide; D008727:Methotrexate