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Objective: To evaluate the clinical characteristics and outcome of NK/T-cell lymphoma 'nasal type' developed in Italian patients., Patients: Between 1997 and 2004, 26 new cases of NK/T-cell lymphoma 'nasal type' were diagnosed in 10 Italian Hematology institutions., Results: All patients were Caucasian, male/female ratio was 19/7, with a median age of 50 years (range 20-80). In 23 cases presentation at the onset was in the nasal cavity or adjacent structures, in two cases the lymphoma onset with skin lesions was followed successively by rhynopharyngeal dissemination, while the remaining case had bone marrow and lymph node involvement followed by oro-pharyngeal involvement. Regarding the stage of disease: 12 patients were in stage I; six in stage II; eight in stage IV. Diagnosis was based on the finding of a NK/T-cell phenotype at the histological and immunophenotypic examination of oropharyngeal or cutaneous lesions. All patients but one were treated with chemotherapy, alone in nine cases or associated to radiotherapy in 14 cases; two patients had chemotherapy, radiotherapy and surgery, while one patient underwent only surgery. Chemotherapy was anthracycline-based in 17 out of 25 cases. In those patients in whom radiotherapy was performed, radiation dosages ranged between 36 Gy and 47.5 Gy, with a median dosage of 40 Gy. Nine patients (34%) were responsive to the treatments: six patients obtained a complete remission and other three a partial remission. The remaining 17 patients resulted refractory or presented a limited response to therapy. The median disease-free survival was 14 months and the median overall survival time was 9 months., Conclusion: The results of this retrospective survey confirmed that NK/T-cell lymphoma 'nasal type' is a very rare lymphoma in the Italian population, and it is characterized by a very bad prognosis. Due to the rarity of this disease, a standardized therapeutic approach is lacking. More data are needed to know the epidemiology of this kind of lymphoma in Europe.

Background: Acute Myelogenous Leukemia (AML) is a common disease in people aged>60 years. About 50% of the patients are not eligible for aggressive chemotherapy (CT) and are only managed with conservative approaches. Results in this subset of patients have not been reported so far., Patients and Methods: We retrospectively evaluated 244 consecutive elderly AML patients (M/F 143/101, median age 72 years, range 60-90) diagnosed at our institution from January 1989 to December 1998 and not eligible for intensive CT. Eighty-nine patients (36.5%) had evolved from previous myelodysplasia (sAML). Fifty-three out of 192 (26.4%) patients with available bone marrow (BM) analysis had oligoblastic leukaemia (blasts<40% and WBC<15x10(9)/l)., Results: Sixty-seven patients (27.5%) were managed with supportive treatment only. One hundred seventy-seven patients (72.5%), in order to control disease, received conservative CT, consisting of Hydroxyurea (HU) (127 patients, 71.7%), Cytarabine and 6-Thioguanine (39 patients, 22%) or low-dose cytarabine (11 patients, 6.3%). Median overall survival was 179 days (1-3278) with 50 patients (20.5%) surviving>12 months. Older age (>75 years), poor WHO PS (>2), lower PLT levels (<50x10(9)/l) and higher absolute peripheral blast count (>5x10(9)/l) showed a negative prognostic impact on survival in multivariate analysis., Conclusions: Our data outline the great heterogeneity of elderly AML patients not eligible for intensive CT. A simple scoring system including easily evaluable parameters, which could distinguish subjects with different prognosis, is proposed. Moreover, randomized studies in order to establish best conservative approaches are warranted.

Objective: To evaluate in a multicenter retrospective study, the clinical and laboratory characteristics and the outcome of patients with acute myeloid leukemia (sAML) previously diagnosed with breast cancer (BC) among an adult acute leukemia population., Patients and Methods: Between June 1992 and July 1996, 3934 new cases of adults with acute leukemia were recorded in GIMEMA Archive of Adult Acute Leukemia (2964 AML, 901 ALL, 69 acute leukemia expressing both myeloid and lymphoid surface markers)., Results: Two hundred patients (5.1%) presented with a history of previous malignancy (21 of them were affected by ALL and 179 by AML). Among sAML, 37 patients (29%) had a previous breast cancer. They consisted of 36 females and 1 male, median age 56 years, range 34-87. The median latency between the 2 malignancies was 54 months (range 5-379). Twenty-seven patients received chemo- and/or radiotherapy for breast cancer (7 only chemotherapy, 6 only radiotherapy, and 14 combined treatment). All patients were surgically treated but in 10 patients surgical debridement was the sole therapy for breast cancer. The drugs most frequently employed were alkylating agents (18 patients), topoisomerase II inhibitors (9 patients), antimetabolites (20 patients) (CMF, CEF and MMM combinations). At onset of sAML the median WBC count was 7.7 x 10(9)/l (0.8-153) and the median platelet count was 33.5 x 10(9)/l (3-305). Considering morphological features, FAB subtypes were 4 M0, 5 M1, 11 M2, 5 M3, 8 M4, 3 M5, and 1 M6. Cytogenetic study was performed on 28 patients and 12 of them presented abnormalities. It is noteworthy that chromosome 5 or 7 abnormalities (typically observed in those patients treated with alkylating agents) were present only in three cases. Thirty-four patients received chemotherapy for sAML, and twenty-five of them achieved a CR (74%), with a median duration of twenty-eight weeks (5-280+). The overall survival was 8 months (1-80+)., Discussion: The high number of sAML we observed in patients with a previous breast cancer, may be due to the fact that this malignancy is the most frequent neoplasm in women and by the high probability of cure with a consequent long disease-free survival. Our results suggest that the risk of sAML after recovery from breast cancer is increasing due to the rise in the number of patients cured from breast cancer, and in the future could be a relevant problem for haematologists.

Background: To evaluate the role and toxicity of ATRA therapy in newly-diagnosed APL patients aged > 60 yrs, the outcome of 16 consecutive elderly APL patients observed between January 1990 and June 1996 were analyzed., Patients and Methods: Their median age was 65.5 yrs (range 60-81 years), the male/female ratio was 7:9, and molecular biology analysis showed a PML/RARa rearrangement in all patients. Seven patients had a concomitant cardiovascular disease. ATRA 45 mg/sqm/day was given to all patients, and in 11 was associated with idarubicin (AIDA protocol); in two patients ATRA was associated with mitoxantrone + ara-C, while the remaining three patients received ATRA alone., Results: Fourteen patients (87.5%) achieved CR, and two patients (12.5%) died during induction. Despite the high CR rate, eight episodes of severe cardiovascular complication were observed in seven patients, three of whom had previously had cardiovascular disease; in addition, three patients had sepsis (two bacterial and one fungal). As of 31 March 1997, 9 of 14 patients were still in first CR after a 19-month (range 7-64 months) median follow-up since attainment of the CR. One patient died in CR of a fungal complication and four patients relapsed after 8, 9, 23 and 35 months following CR: two of them achieved a second CR lasting seven and +15 months with ATRA alone. Of the nine patients still in first CR, only three have received the planned consolidation therapy and five have been in CR for more than 24 months (+25, +33, +34, +38, +63)., Conclusions: Despite the fact that most of these patients received shorter consolidation treatments than do younger patients, the good results achieved in them might be considered an indication for modifying treatment schedules in order to reduce severe toxicity and improve protocol compliance.

Background: Carboplatin is a second-generation platinum compound that in combination with etoposide and intermediate doses of cytarabine, in early clinical trials has demonstrated high efficacy in refractory acute myelogenous leukemia and the blastic phase of CML., Patients and Methods: Starting in April 1992, 17 consecutive patients with the blastic phase (BP) of chronic myelogenous leukemia (CML) and a median age of 33 years (range 10 to 45) received a new treatment regimen consisting of etoposide (100 mg/m2/d i.v. over one hour), intermediate-dose cytarabine (500 mg/m2/d i.v. over one hour, q12 hours) and carboplatin (150 mg/m2/d continuous infusion) on days 1 3 and 8 10 (VAC regimen). The BP phenotype was myeloid in 16 patients and hybrid in one. At the time of their BP diagnoses, two patients showed extramedullary involvement, in eight patients the BP was preceded by an accelerated phase., Results: Overall, 11 of 17 patients (65%) achieved complete remission and 7 of 11 responding patients (64%) manifested a cytogenetic conversion ranging from 38% to 100% Ph-negative metaphases: one patient died in CR of hemothorax, three died of sepsis during induction therapy and three patients (17.5%) had resistant disease. As of April 1996, four patients are alive, three of them in first remission at +7, +10, +16 months after CR, and one in BP at +38 months after the start of VAC therapy. Profound myelosuppression was observed in all patients; extrahematologic toxicity, especially involved the gastro-intestinal tract, with grade > 2 mucositis in five patients (29.5%) and liver dysfunction in five (29.5%). No renal toxicity or ototoxicity was observed., Conclusions: Despite the brief relapse-free duration, the high remission rate and tolerable toxicity indicate that the VAC combination chemotherapy has a high antileukemic efficacy, and warrants further evaluation for the treatment of CML in acute phase, provided a post-remission BMT strategy is feasible.

Background: Various polichemotherapy regimens, including either high- or intermediate-dose Ara-C, are generally utilized to reinduce remission in relapsed AML patients. After achieving second CR, bone marrow transplantation (either allogeneic or autologous) represents the treatment of choice for eligible patients, with the aim of prolonging remission duration and improving disease-free survival., Patients and Methods: Fifty AML patients in first hematological relapse were treated with MEC regimen, consisting of a 6-day induction cycle [mitoxantrone 6 mg/m2/day, cytarabine (Ara-C) 1 g/m2/day and VP-16 80 mg/m2/day] followed by a 4-day cycle with the same drugs for patients achieving complete remission (CR); allogeneic or autologous bone marrow transplantation (BMT) were planned as post-consolidation treatment., Results: Thirty-four patients (68%) achieved second CR, 3 (6%) died during induction and 13 were refractory. CR rate was significantly higher in patients with a first CR lasting > 6 months (82% vs. 41%, P < 0.001). Out of the 34 patients in CR after the 4-day cycle, 18 (53%) were not eligible to transplant and did not receive any further therapy and 16 (47%) received autologous (15 cases) or allogeneic (1 case) BMT at a median time of 2 months from second CR. Twenty-two patients relapsed after a median time of 6 months (range 1-31), 1 patient died from transplant-related toxicity and 11 are in continuous CR [7 out of 16 (44%) in the transplanted and 4 out of 11 (36%) in the non-transplanted group]. Overall survival and event-free survival for the 50 patients were 29% and 19% at 70 months, respectively. The disease-free survival for the 34 patients who obtained second CR is 29% projected at 69 months [41% at 69 months for 16 transplanted patients versus 18% at 49 months for the remaining 18 patients (P = 0.007)]., Conclusions: These results show that MEC followed by high-dose post-consolidation treatment is a promising approach in relapsed AML; however, alternative strategies are to be investigated for the relevant fraction of patients that, even achieving second CR, are not eligible for BMT.