Myelodysplastic syndromes (MDS) include a group of heterogeneous hematological disorders with a variable risk of leukemic evolution and short survival. Around 40-50% of patients show abnormal karyotypes that are mostly characterized by monosomies or deletions. Cytogenetic findings are an independent prognostic factor and the International prognostic scoring system (IPSS) differentiates three cytogenetic categories, despite the Intermediate one being heterogeneous. The aim of this study, including 421 Argentinean patients with primary MDS, is to characterize the cytogenetic profile, to test its prognostic value and to compare partial and monosomal karyotypes against other cytogenetic findings. An abnormal karyotype (median survival: 26 months) was observed in 176 patients. The presence of complex karyotypes, number of alterations, and the IPSS cytogenetic groups showed significant differences for predicting outcome. Behavior of patients with isolated deletions (median survival: 49 months) did not differ from those with normal karyotype (56 months, P 5 0.654) or Good prognostic findings (43 months, P 5 0.371). However, a worse prognosis was observed when another alteration was added (31 months, P 5 0.043). Karyotypes with autosomal monosomies (median survival: 16 months) had a prognostic impact similar to other Poor cytogenetic findings (17 months, P 5 0.626). In our population classified according to French-American-British (FAB) or World Health Organization (WHO), this new categorization of cytogenetic abnormalities, recognizing three different risk groups, showed an independent prognostic impact and a better discriminating power than the IPSS categories. It can be concluded that all isolate deletions (excluding 7q-) are good prognostic findings and all monosomies (excluding Y chromosome loss) are bad indicators. Am. J. Hematol. 86:540-545, 2011. V

Anaplastic lymphoma kinase–negative anaplastic large cell lymphoma (ALK– ALCL) is an aggressive neoplasm of T-cell/null-cell lineage. The T-Cell Project is a global prospective cohort study that consecutively enrolled patients newly diagnosed with peripheral T-cell lymphoma, registered through a centralized computer database between September 2006 and February 2018. Of 1553 validated cases from 74 sites in 13 countries worldwide, 235 were reported as ALK– ALCL. The median age at diagnosis was 54 years (range, 18-89 years), with a male predominance (62%). Stage III to IV disease was identified in 71% of patients, bulky disease and bone marrow involvement were uncommon, and 66% of patients presented with a low (0-1) International Prognostic Index score. Of all treated patients, 85% received multiagent initial chemotherapy, and 8% were consolidated with autologous hematopoietic cell transplantation. The initial overall and complete response rates were 77% and 63%, respectively. After a median follow-up of 52 months (95% confidence interval [CI], 41-63), the median progression-free survival (PFS) and overall survival (OS) were 41 months (95% CI, 17-62) and 55 months (95% CI, 36-75), respectively. The 3- and 5-year PFS rates were 52% and 43%, and the 3- and 5-year OS rates were 60% and 49%. Treatments containing both anthracycline and etoposide were associated with superior OS (P = .05) but not PFS (P = .18). In this large prospective cohort study, outcomes comparable to those previously reported in the retrospective International Peripheral T-Cell Lymphoma Project were observed. The study underscores the need for introducing novel platforms for ALK– ALCL and establishes a benchmark for future clinical trials. This trial was registered at www.clinicaltrials.gov as #NCT01142674.

PURPOSE Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease caused by the human T-cell leukemia virus type 1. Real-world data of ATLL in Latin America are lacking. PATIENTS AND METHODS We analyzed patients with ATLL (acute, lymphomatous, chronic, and smoldering) encountered in 11 Latin American countries between 1995 and 2019. Treatment response was assessed according to the 2009 consensus report. Survival curves were estimated using the Kaplan-Meier method and log-rank test. RESULTS We identified 253 patients; 226 (lymphomatous: n = 122, acute: n = 73, chronic: n = 26, and smoldering: n = 5) had sufficient data for analysis (median age 57 years). Most patients with ATLL were from Peru (63%), Chile (17%), Argentina (8%), and Colombia (7%). Hypercalcemia was positively associated with acute type (57% v lymphomatous 27%, P = .014). The median survival times (months) were 4.3, 7.9, 21.1, and not reached for acute, lymphomatous, chronic, and smoldering forms, with 4-year survival rates of 8%, 22%, 40%, and 80%, respectively. First-line zidovudine (AZT)-interferon alfa (IFN) resulted in an overall response rate of 63% (complete response [CR] 24%) for acute. First-line chemotherapy yielded an overall response rate of 41% (CR 29%) for lymphomatous. CR rate was 42% for etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone versus 12% for cyclophosphamide, vincristine, doxorubicin, and prednisone–like regimen ( P < .001). Progression-free survival at 1 year for acute type patients treated with AZT-IFN was 67%, whereas 2-year progression-free survival in lymphomatous type patients who achieved CR after chemotherapy was 77%. CONCLUSION This study confirms Latin American ATLL presents at a younger age and has a high incidence of lymphomatous type, low incidence of indolent subtypes, and worse survival rates as compared with Japanese patients. In aggressive ATLL, chemotherapy remains the preferred choice for lymphomatous favoring etoposide-based regimen (etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone), whereas AZT-IFN remains a good first-line option for acute subtype.

In our series, more than half of the patients achieved long term OS with AHSCT. Less toxic conditioning regimens or more intensive GVHD prophylaxis could lead to better results in some children.

No abstract

No abstract

Adult T-cell leukemia/lymphoma (ATLL) is a largely incurable disease. Cutaneous involvement is common and could be first symptom of the disease. We analyzed 169 patients with ATLL of whom 63 had cutaneous involvement. Cutaneous involvement was found in 48, 27, 17, and 60% of acute, lymphomatous, chronic and smoldering ATLL cases, respectively. Eight cases had primary cutaneous tumoral variant. Erythroderma (24%) and plaques (22%) were the most frequent skin lesions. The presence of cutaneous involvement was associated with better overall survival compared to non-cutaneous involvement (aHR 0.55 [95% CI: 0.37-0.82], p < 0.01; 1-year OS 53 vs. 27%, respectively, p ¼ 0.012). Combination zidovudine and interferon-alpha (AZT-IFN) yielded high response rates (overall response, OR ¼ 100%, n ¼ 8; complete response 62.5%) compared to chemotherapy (OR ¼ 33.3%, n ¼ 12/36). In conclusion, cutaneous involvement was associated with better survival in Latin American patients with ATLL. AZT-IFN demonstrated encouraging responses in ATLL patients with cutaneous involvement.

Introduction: Anaplastic Large Cell Lymphoma, ALK-Negative(ALCL, ALK-) is an aggressive lymphoid neoplasm of T/null-cell lineage with strong expression of CD30, which represent 5.5% of all PTCL and NKTCL, with a unique ethnic and geographical distribution. ALCL, ALK-displays a poorer outcome compared to ALK-positive, with a 5-year OS of 49% and 70%, respectively. This study aims to provide accurate and update information on prognostic factors, therapy, and outcomes of ALK-ALCL registered in the T-Cell Project.Methods: Eligible patients (pts) with the first diagnosis of aggressive, mature PTCLs were prospectively registered at a dedicated website and baseline demographic, clinical and laboratory data, as well as therapy details and outcome data were collected. Results: From Sept 2006 to Feb 2018, a diagnosis of ALCL, ALK-was reported in 235 cases out of 1553 validated cases registered by 77 sites from 14 countries worldwide. The median age at diagnosis was 54 (range, 18-89) years with male predominance (61%). Stage III-IV disease was identified in 71% pts, bulky and bone marrow involvement were uncommon. Therapy was administered according to treating physician's standard practice. Out of 205 patients for which we have available data about treatment, in 9% were addressed to consolidating post-transplant chemotherapy.The overall response rate was 77% with 57% of patients achieving complete remission. After a median follow-up of 55 (95% CI, 36-75) months, the PFS was 41 (95% CI, 17-62) months, the 3-year and 5-year PFS rates were 47% and 39%, respectively. The median OS was 55 (95% CI, 36-75) months, 3-year and 5-year OS rates were 60% (95CI 48-72) and 49% (95CI35-59), respectively. International Prognostic Index (IPI) and the Prognostic Index for T-cell Lymphomas (PIT) were both predictive of clinical outcomes. Treatment with anthracycline and etoposide vs. anthracycline-only containing regimens was associated with a better OS( 3 and 5-year OS 56% and 44% vs 76% and 69%, respectively, (P = 0.05), but not PFS 3 and 5 year PFS 47% (95CI 32-59) and 39 % (95CI 29-48) vs. 65% (95CI 32-87) and 50% (95CI 36-89) respectively (P = 0.186).In the multivariate analysis the presence of B symptoms (P = 0.008), elevated LDH rate (P = 0.001), ECOG-PS >2 (P = 0.001) andPTL<150x10 9 (P = 0.05), were significant for OS. The presence of B symptoms (p = 0.02), elevated LDH (P = 0.001) and ECOG-PS>2 (P = 0.001) maintained their adverse prognostic value for PFS.Conclusions: ALCL, ALK-remains a difficult T cell lymphoma to be cured. Combinations regimens that include both etoposide and anthracycline might improve outcomes. In this large prospective ABSTRACT 283 cohort study, we observed an outcome comparable with a previous report from the retrospective International Lymphoma Project. The study underscores the urgent need for designing new treatment platforms for ALCL, ALK-, and establishes a benchmark for future prospective clinical trials.Background: Mycosis fungoides (MF) is a rare, generally indolent non-Hodgkin T-cell lymphoma of ...