Histological transformation (HT) to diffuse large B-cell lymphoma (DLBCL) is a rare and poorly reported complication of Waldenström macroglobulinaemia (WM). We performed a retrospective study of 77 WM patients with biopsy-proven transformation to DLBCL. The median time from WM diagnosis to HT was 4·6 years and 16 patients (21%) had never been treated for WM. At HT, extranodal sites were observed in 91% of patients with a rather high incidence of central nervous system, cutaneous or testicular involvement. Fluorodeoxyglucose-positron emission tomography was performed in half of the patients and the median maximum standardized uptake value was 15 for transformed disease. More than 80% of cases with available data for assessment by the Hans' algorithm harboured a non-germinal centre B-cell phenotype. First-line treatment for transformation consisted of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)-like regimen in 85% of patients. The overall response rate after first-line treatment was 61% and the median overall survival was only 16 months for the entire cohort. Time to transformation above 5 years (P = 0·0004) and elevated LDH (P = 0·02) were associated with worse outcome. Based on these findings, HT should be considered and lead to a biopsy in WM patients presenting with extranodal involvement, elevated LDH and constitutional symptoms. The optimal therapeutic approaches remain to be defined.
Despite progress in the understanding of leukemia pathophysiology, the treatment of acute myeloid leukemia (AML) remains challenging. In patients with refractory or relapsed (R=R) AML, the prognosis is still poor and this group is targeted for new drug development. We reviewed the outcome of 47 patients, with R=R AML after at least one course of intensive chemotherapy, treated with 5-azacytidine in three different French institutions. The overall response rate was 38% including complete remission in 21%, partial remission in 11%, and hematological improvement in 6% of cases. Median time to relapse was 6 (range, 1-39) months. Median overall survival was 9 months (not reached by responders vs. 4.5 months for nonresponders patients, P 5 0.0001). Univariate analysis identified the absence of peripheral blood blasts and <20% bone marrow blasts as prognostic factors for both overall response and survival, but not age, ECOG=PS, type of AML, cytogenetic, status of the disease, number of previous lines of therapy, previous hematological stem cell transplantation, or white blood cells count. Bone marrow blasts percentage <20% was the only independent prognostic factor identified by multivariate analysis for overall response (P 5 0.0013) and survival (P 5 0.0324). Six patients in remission could proceed to an allogenic hematological stem cell transplantation. The drug-related grade 3=4 adverse events were hematopoietic toxicities (38%) and infection (32%). In conclusion, this study suggests that a salvage therapy with 5-azacytidine is an interesting option for patients with R=R AML after intensive chemotherapy. Prospective randomized studies are needed to demonstrate a superiority of this approach over others strategies. Am. J. Hematol. 88:601-605,
© F e r r a t a S t o r t i F o u n d a t i o nto report on the therapeutic characteristics of these patients as well as on the role of high-dose therapy and autologous stem cell transplantation in these settings. Methods Study designPatients with histologically confirmed diagnosis of nonHodgkin lymphoma (NHL) made between May 2002 and January 2012 with concomitant systemic and neurological involvement were retrieved from the database of the participating centers, from The Lymphoma Study Association (LYSA) database and the French network for oculocerebral lymphoma (LOC).In accordance with the Declaration of Helsinki, approval of the University Hospital of Amiens Nord-Ouest II Ethics Committee was obtained. Patient eligibilityPatients 18 years or older with an sNHL diagnosis with nervous system involvement were eligible for this study. Diagnosis of NHL was made according to international diagnostic criteria used at the time of diagnosis. Diagnosis of systemic lymphoma was done on lymph nodes or using tissue biopsies. Nervous system involvement was defined by brain parenchyma, intra-ocular, cranial nerve, or meningeal involvement, as well as paravertebral mass and CSF involvement with lymphoma cells. Diagnosis was based on positive cerebrospinal fluid (CSF) cytology or immunophenotyping or by biopsy-proven parenchymal brain localization. Patients with CNS localization based on a typical CT-scan and/or magnetic resonance imaging (MRI) and concurrent sNHL were included as well. Treatment and assessmentsAt base-line assessments of patients and disease included ECOG score, Ann Arbor stage, international prognostic index (IPI), lactate dehydrogenase (LDH) level, bone marrow histology, CSF cytological and/or flow cytometry analysis, CT-scan of the thorax, abdomen and pelvis. Cerebral and vertebral CT-scan and/or MRI were also recorded. G. Damaj et al. 1200haematologica | 2015; 100(9) marrow, bones, spleen, liver, breast, testis, adrenal glands, ovaries, gastro-intestinal tract, lungs and parotid gland; intraventricular, parenchymal, meningeal, cranial © F e r r a t a S t o r t i F o u n d a t i o nThe type of chemotherapy administered, total dose of drugs per cycle and the use of high-dose therapy as well as radiotherapy were retrieved from the original patient files.Response assessment was performed by the on-site referent physician according to the International Working Group Criteria (IWC) for sNHL and the International Primary CNS Lymphoma Collaborative Group for CNS disease. 7,8 Statistical analysisQuantitative variables were expressed as mean±standard deviation or as median [range] and qualitative variables as percentages. The Student or Wilcoxon test was used for comparison of quantitative variables between two groups. χ 2 or Fisher's test were used to compare categorical variables. Overall survival (OS) was defined as the time between diagnosis and the date of death or last follow up. Univariate standard and time-varying (for intensive therapy) Cox models were used to assess prognostic factors for...
Multiple Myeloma (MM) is rare in young patients - especially before 40 years at diagnosis, representing less than 2% of all patients with MM. Little is known about the disease characteristics and prognosis of these patients. In this study we examined 214 patients diagnosed with MM ≤ 40 years old over 15 years, in the era of modern treatments. Among them, 189 patients had symptomatic MM. Disease characteristics were similar to older patients: 35% had anemia, 17% had renal impairment, and 13% hypercalcemia. The staging was ISS-1 in 52.4%, ISS-2 in 27.5% and ISS-3 in 20.1%. Overall, 18% of patients had high risk cytogenetics (del 17p and/or t(4;14)). Ninety percent of patients received intensive chemotherapy followed by autologous stem cell transplant, and 25% of patients had allogeneic stem cell transplantation predominantly at time of relapse. The median follow-up was 76 months, the estimated median overall survival was 14.5 years and the median PFS was 41 months. In multivariate analysis, bone lesions (HR=3.95; p=0.01), high ISS score (HR=2.14; p=0.03) and high-risk cytogenetics (HR=4.54; p<0.0001) were significant risk factors for poor outcomes. Among predefined time-dependent covariables, onset of progression (HR=13.2; p<0.0001) significantly shortened OS. At 5 years, Relative Survival compared to same age and sex matched individuals was 83.5%, and estimated Standardized Mortality Ratio was 69.9 (95%CI 52.7-91.1), confirming that MM dramatically shortens the survival of young patients despite an extended survival after diagnosis.
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