{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,6]],"date-time":"2025-12-06T04:52:00Z","timestamp":1764996720519},"reference-count":33,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2003,8,25]],"date-time":"2003-08-25T00:00:00Z","timestamp":1061769600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2003,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Although its cell of origin is still controversial, the blastic NK\u2010cell 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\u2010organ disease. Consistently effective treatments have not been developed for this malignancy. The present report describes two elderly patients with widespread blastic NK\u2010cell 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\u2010cell receptor (TCR) \u03b3 chain gene rearrangement. The cells also exhibited a low CD45 expression and strong CD99 (mic\u20102) expression, as seen in immature lymphoid malignancies. The above findings support the precursor NK\u2010cell, rather than mature NK\u2010 or non\u2010NK\u2010cell, origin of the malignant cells. It is noteworthy that the two patients achieved complete responses to treatment with hyper\u2010CVAD (fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) alternating with high\u2010dose methotrexate\/cytarabine, a regimen currently utilized in acute lymphoblastic leukemia and high\u2010grade 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\u2010CVAD regimen to other therapeutic strategies may be warranted. Am. J. Hematol. 74:46\u201351, 2003. \u00a9 2003 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.10381","type":"journal-article","created":{"date-parts":[[2003,8,27]],"date-time":"2003-08-27T13:39:22Z","timestamp":1061991562000},"page":"46-51","source":"Crossref","is-referenced-by-count":31,"title":["Complete remission in advanced blastic NK\u2010cell lymphoma\/leukemia in elderly patients using the hyper\u2010CVAD regimen"],"prefix":"10.1002","volume":"74","author":[{"given":"Michael","family":"Shapiro","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mariusz A.","family":"Wasik","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacqueline M.","family":"Junkins\u2010Hopkins","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alain H.","family":"Rook","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carmela C.","family":"Vittorio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haruka","family":"Itakura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew C.","family":"Frankel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sofia","family":"Georgala","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen J.","family":"Schuster","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2003,8,25]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V82.1.1.bloodjournal8211"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0145-2126(98)00194-5"},{"key":"e_1_2_1_4_2","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1200\/JCO.2000.18.3.547","article-title":"Results of treatment with Hyper\u2010CVAD, a dose\u2010intensive regimen, in adult acute lymphocyte leukemia","volume":"18","author":"Kantarijian HM","year":"2000","journal-title":"J Clin Oncol"},{"key":"e_1_2_1_5_2","first-page":"11a","article-title":"Outcome with the Hyper\u2010CVAD regimen in lymphoblastic lymphoma (LL)","volume":"18","author":"Thomas D","year":"1999","journal-title":"Proc Am Soc Clin Oncol"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V89.12.4501"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/hon.2900130102"},{"key":"e_1_2_1_8_2","first-page":"28","article-title":"Inconsistent association of Epstein\u2010Barr virus with CD56 (NCAM)\u2010positive angiocentric lymphoma occurring in sites other than the upper and lower respiratory tract","author":"Kobashi Y","year":"1996","journal-title":"Histopathology"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2559.1998.00571.x"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1159\/000018383"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199503000-00006"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.2000.03489.x"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1159\/000040955"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/110.4.478"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199710000-00014"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V99.5.1556"},{"key":"e_1_2_1_17_2","first-page":"214","volume-title":"World Health Organization classification of tumours. 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