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Patients were classified according morphologic, immunophenotypic, and cytogenetic findings. Treatment protocols were similar between centers. For patients up to 60 years of age, the treatment protocol was adapted according to performance status and the presence of comorbidities (in particular, cardiac disorders). Briefly, the conventional chemotherapy consisted of daunorubicin (45-90 mg/m 2 per day for 3 days) and cytarabine (100-200 mg/m 2 per day for 7 days) or thioguanine, cytosine arabinoside, and daunorubicin as induction, 1 followed by 3 or 4 cycles of consolidation therapy with high doses of cytarabine (.1 g/m 2 per day). For patients who did not achieve complete remission (CR) after 1 course of chemotherapy, a second course was administered between days 28 and 35 after the end of the first course. CR was assessed by bone marrow examination on day 28 after each course of chemotherapy. For those who needed it, a postremission therapy based on autologous or allogeneic transplantation was performed. Patients older than 60 years were treated with low-dose cytarabine; a combination of etoposide, thioguanine, and idarubicin; or best supportive care. The local research ethics board of each participating center approved the study. Research was conducted in accordance with the Declaration of Helsinki.The baseline characteristics are summarized in supplemental Table 1. One hundred thirty patients were enrolled in Recife (northeast Brazil, 54%), and 111 patients were enrolled in Campinas (southeast Brazil, 46%). Baseline features were similar between centers. The median age was 47 years (range, 18-97 years) with 114 males (47%). Sixty-two patients (26%) were older than 60 years. Pretreatment bone marrow samples were analyzed by G-banding cytogenetics, of which 187 (78%) were successful. According to Medical Research Council trials, 2 patients were stratified as follows: favorable (30/187, 16%), intermediate (119/187, 64%), and adverse (38/187, 20%). Overall, 101 patients (42%) were cytogenetically normal. To test whether the samples without cytogenetics results were missing at random, the overall survival (OS) was evaluated for patients with and without cytogenetics data. The 5-year OS rate did not differ between patients with (18%) and without (23%) available cytogenetics data (P 5 .372). Additionally, the entire cohort was fully characterized for NPM1 and FLT3-ITD mutations. Details can be found in the supplemental Data, available on the Blood Web site.Out of 241 enrolled patients, 39 patients (16%) who started the induction treatment were lost to follow-up without assessment for CR. Of 202 evaluable patients, 115 (57%) achieved complete hematologic remission. CR rates according to the cytogenetic risk stratification were 33%, 64%, and 77% for adverse, intermediate, and favorable groups, respectively (P 5 .001). The logistic regression analysis revealed that age (odds ratio [OR], 0.95; 95% confidence interval [CI], 0.93-0.98; P 5 .002) and cytogenetic risk stratification (OR, 0.41; 95% CI, 0.19-0.89; P 5 .024) wer...

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88% (46/52) of the patients with PV, 47% (39/81) with ET, and 77% (8/11) with PMF were positive for JAK2 V617F, while more than 35% of the individuals were JAK2 V617F-negative, confirming a high prevalence of this abnormality in MPNs, more frequently with a low mutated allele burden, similar to what has been reported in other Western countries, despite differences among methods used to detect this mutation. Screening for JAK2 V617F may allow specific management of these diseases with JAK2 inhibitors in the future and highlights the need for further studies on the pathogenesis of BCR-ABL-negative JAK2 V617F-negative MPNs.

BackgroundInfectious complications represent the main cause of morbidity and mortality in chronic lymphocytic leukemia. It has been reported that polymorphisms of the mannose-binding lectin 2 (MBL2) genes are correlated with MBL protein serum levels and, consequently, are associated with the development of infectious diseases.ObjectiveThe purpose of this study was to investigate the possible association between MBL2 gene polymorphisms and risk of infection in chronic lymphocytic leukemia patients.MethodsPeripheral blood samples from 116 chronic lymphocytic leukemia patients were collected; after genomic DNA extraction, real time polymerase chain reaction was used to determine the polymorphisms of the promoter region and exon 1 of the MBL2 gene.ResultsA high frequency of Binet stage A (p-value = 0.005) and absence of splenomegaly (p-value = 0.002) were observed in patients with no infection; however, variant alleles/ genotypes and haplotypes of this gene had no impact on the risk of infection.ConclusionTo the authors’ knowledge, this is the first study describing the association between MBL2 polymorphisms and infectious disease in chronic lymphocytic leukemia. Although it was not possible to demonstrate any influence of MBL2 polymorphisms as a genetic modulator of infection in chronic lymphocytic leukemia, the authors believe that the present data are clinically relevant and provide the basis for future studies.© 2014 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. All rights reserved.

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