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Background: Acute promyelocytic leukaemia (APL) is a subtype of acute myeloid leukaemia with high curability rates. However, it is often accompanied by severe coagulopathy and bleeding risk and thus represents a potentially fatal haematological emergency requiring immediate treatment. Spontaneous splenic rupture is a rare event in all haematological malignancies. Only two clinical cases have been described so far in a setting of APL. Case report: We report a patient with APL without preceding splenomegaly who underwent urgent splenectomy for spontaneously occurring splenic rupture during induction chemotherapy. After surgery the patient completed induction chemotherapy and achieved complete remission. Conclusion: This is the second case of spontaneous splenic rupture without preceding splenomegaly in a patient with APL during induction chemotherapy described so far. Our case demonstrates that emergent splenectomy can be lifesaving even in the unfavourable condition of patient with severe immune deficiency.

Aims. This study compares the outcomes of patients with high-risk acute myeloid leukemia (AML) who underwent allogeneic stem cell transplantation (SCT) after conditioning combining busulfan (16 mg/kg orally) and cyclophosphamide (120 mg/kg intravenously) (BU-CY) with those allografted after administration of fludarabine (150 mg/m 2 intravenously), busulfan (12 mg/kg orally) and thymoglobulin (6 mg/kg intravenously) (FLU-BU12-TG). Material and methods. SCT after BU-CY and FLU-BU12-TG was performed in 21 and 10 AML patients. There were no significant differences between groups in number of patients treated in complete disease remission, gender, age, donors, CD34+, mononuclear cell (MNC) count in the graft and follow-up period. However, significantly more SCTs from unrelated (90% vs. 19%; p=0.00018) and HLA-mismatched donors (50% vs. 0%; p=0.0004) were performed in the FLU-BU12-TG group. The Cox proportional hazards model was used to assess the risk of post-transplant AML relapse and non-relapse mortality (NRM). The probability of post-transplant 2-year event-free survival (EFS) and overall survival (OS) were calculated using the Kaplan-Meier method. Results. No significant differences were found between the FLU-BU12-TG and BU-CY groups in risk of AML relapse (HR=1.036; 95% CI [0.102 - 10.47]; p=0.9), post-transplant NRM (HR=0.25; 95% CI [0.031 - 1.96]; p=0.18), 2-year EFS (89% vs. 43%; p=0.19) or OS (79% vs. 57%; p=0.23). Conclusion. These pilot results demonstrate the efficacy of the new FLU-BU12-TG conditioning regimen in patients allografted for high-risk AML. This conditioning might become an alternative approach in patients at high risk of severe post-transplant complications after the standard BU-CY myeloablative regimen.

Background. Diffuse large B-cell lymphoma (DLBCL) accounts for up to 40% of all non-Hodgkin’s lymphomas diagnosed in the western hemisphere. Determination of the gene expression profile has confirmed the physiological heterogeneity of the disease and defined three molecular prognostic subgroups – germinal center B-cell-like (GCB), activated B-cell-like (ABC) and primary mediastinal B-cell lymphoma (PMBL) – with different gene expression and prognosis. Methods and Results. This review covers current knowledge on the most frequent recurrent cytogenetic and molecular cytogenetic aberrations in molecular DLBCL subgroups. Conclusions. Cytogenetic and molecular cytogenetic techniques used to determine nonrandom chromosomal aberrations in patients with DLBCL have revealed the incidence of frequent cytogenetic aberrations in the subgroups reported, suggesting their potential use for more accurate prognostic stratification of DLBCL, contributing to personalized selection of the most effective therapy.

Aims: Chronic lymphocytic leukemia (CLL) is the most common adult leukemia with a very heterogeneous course. Progress in molecular genetic characterization of CLL has confirmed the prognostic role of unbalanced chromosomal abnormalities currently defined by molecular cytogenetic methods: conventional karyotyping and FISH. However, a significant percentage of genomic abnormalities escapes routine investigation due to the limitations of these methods. It is presently clear that some of these aberrations have impact on prognosis and disease progression. Methods: We examined copy number changes in the tumor genomes of 50 CLL patients using bacterial artificial chromosome (BAC) and/or oligonucleotide array platforms. We compared the results of arrayCGH with those obtained by FISH and conventional cytogenetics and evaluated their clinical importance. Results: A total of 111 copy number changes were detected in 43 patients (86%) with clonal abnormalities present in at least 23% of the cells. Moreover, 14 patients (28%) were found to have 39 genomic changes that had not been detected by standard cytogenetic and/or FISH analyses. These included possibly prognostically important recurrent 2p and 8q24 gains. The most frequent unbalanced changes involved chromosomes 18, 7, 3, 9 and 17. We also determined the minimal deleted region on chromosome 6q in 7 cases by chromosome 6/7 specific array. Conclusions: The results showed that a subset of potentially significant genomic aberrations in CLL is being missed by the current routine techniques. Further, we clearly demonstrated the robustness, high sensitivity and specificity of the arrayCGH analysis as well as its potential for use in routine screening of CLL.

Background. Follicular lymphoma accounts for about 20–30% of non-Hodgkin’s lymphomas. Clinical behaviour and overall prognosis are highly variable, ranging from indolent forms with occasional spontaneous remissions to rapidly progressive disease. Methods and Results. Modern treatment strategies have shifted from a primarily “palliative” approach to more intensive risk-adapted therapy with the intention of achieving complete long-term remission. New targeted treatment with monoclonal antibodies (MoAb) and radioimmunoconjugates (RIT) has resulted in unprecedented improvements in treatment outcome. At the same time, a large amount of information is now available on lymphomagenesis, the role of the microenvironment of lymphomatous follicles and cytogenetic abnormalities. We can better understand the role of the patient’s innate anti-lymphoma immunity. Although no standard front-line therapy has been established, increasingly more data show that risk-adapted treatment strategy have survival benefits for high-risk patients. For this reason, accurate prognostic indices are urgently needed to find optimal therapies for particular lymphoma patients. Whereas the currently used FLIPI index was established in the pre-rituximab era, the newly designed FLIPI 2 index still needs to be confirmed in prospective trials. Conclusion. New therapeutic approaches with MoAb, RIT and other biological agents allow the population to be divided into increasing numbers of groups with different outcomes. All in all, in the near future, we will probably not use only one basic prognostic index for all populations of FL patients. New prognostic schemes should analyze patients separately and include both disease- and patient/host-related parameters.

Background: Nodal peripheral T-cell lymphomas (PTCLs) are infrequent subtypes of non-Hodgkin’s lymphomas. The WHO classification recognizes three subgroups of nodal PTCL: peripheral T-cell lymphoma not otherwise specified (PTCL, NOS), anaplastic large cell lymphoma (ALCL) and angioimmunoblastic lymphoma (AIL). The clinical course is aggressive and despite multiagent chemotherapy, the median survival is about 2 years. Optimal first-line chemotherapy is not established and the role of high-dose therapy with autologous stem cell support is still controversial. Aim: To analyze the long-term outcome of PTCL patients treated with intensive first-line chemotherapy with highdose therapy and autologous transplant consolidation. Method: Sequential chemotherapy protocol consisting of 3 cycles of CHOEP-21-like regimen (PACEBO), 1 cycle of an ifosfamide and methotrexate-based regimen (IVAM) and a priming regimen with high-dose cytosine arabinoside (HAM). Consolidation was provided with myeloablative conditioning (BEAM 200) and autologous stem cell support. Eighty-four patients with aggressive high-risk lymphoma were treated with the sequential protocol from 2000 to 2007 in our institution. Here we report our experience with 18 patients with nodal PTCL (10 PTCL, NOS; 3 ALCL, ALKnegative; 2 ALCL, ALK-positive; 2 ALCL, unknown ALK status; 1 AIL). Results: Eleven (61 %) patients achieved complete remission, 3 (17 %) partial remission and 4 (22 %) patients failed the procedure. The overall response rate was 77.8 %. After a median follow-up of 25.7 months, nine patients relapsed or progressed (6 PTCL, NOS; 2 ALCL ALK-positive; 1 ALCL ALK-negative; median 14.1 months) and four patients died (lymphoma progression). The relapse was treated with allogeneic stem transplantation in one patient. The 2-year progression-free survival (PFS) was 52 % (95 % CI, 0.27 to 0.76); the 2-year overall survival rate reached 71 % (95 % CI, 0.47 to 0.95). Conclusion: Our results show that intensive first-line chemotherapy with high-dose therapy and autologous transplant consolidation offers a chance for long-term survival in patients with chemosensitive PTCL.