The treatment of patients with chronic myelomonocytic leukemia (CMML) with transplant has not been optimized. We retrospectively reviewed the data for 83 consecutive patients with CMML (47 with CMML-1/2 and 36 with CMML progressed to acute myeloid leukemia) who received an allogeneic stem cell transplant (allo-SCT) at our institution between April 1991 and December 2013 to identify factors associated with improved survival and determine whether treatment with hypomethylating agents before transplant improves progression-free survival (PFS). The median age of the cohort was 57 years. Seventy-eight patients received induction treatment before transplant, with 37 receiving hypomethylating agents and 41 receiving cytotoxic chemotherapy. Patients treated with a hypomethylating agent had a significantly lower cumulative incidence of relapse at 3 years post-transplant (22%) than those treated with other agents (35%; P = .03), whereas treatment-related mortality at 1 year post-transplant did not significantly differ between the groups (27% and 30%, respectively; P = .84). The lower relapse rate resulted in a significantly higher 3-year PFS rate in patients treated with a hypomethylating agent (43%) than in those treated with other agents (27%; P = .04). Our data support the use of hypomethylating agents before allo-SCT for patients with CMML to achieve morphologic remission and improve PFS of these patients. Future studies are needed to confirm these findings.

regulators of cytokine signaling that inhibit cytokine action by inhibiting Jak activation. Of the family members, SOCS-1 and SOCS-3 are the most potent inhibitors of cytokine-induced signals. Forced expression of SOCS-1 or SOCS-3 down-regulates a variety of cytokine signal pathways including IFN-α.8 Recent data indicate that SOCS-2 and SOCS-3 mRNA is constitutively expressed in most blast cells of patients with CML blast crisis but not in the early stage of disease. These results suggest that SOCS-2 and SOCS3 might be involved in unresponsiveness of IFN-α therapy in patients with CML blast crisis. As primary or acquired resistance to IFN-α is often observed in chronic phase CML patients, it is important to determine whether the tumor cells from such patients also show changes in the expression of SOCS, specially SOCS-1 which has never been evaluated in CML. In the present study we demonstrate that SOCS-1 was constitutively expressed in a group of newly diagnosed CML patients and that this overexpression correlated with cytogenetic response to IFN-α.

Bagnall and collegues1 report a technique to investigate an inversion that disrupts the factor VIII (F8) gene and that represents a frequent cause of severe hemophilia A. This large genomic rearrangement, identified for the first time by Brinke et al2 in 2 haemophilic monozygotic twins, affects intron 1 of the F8 gene. Bagnall et al1 demonstrated that this inversion of intron 1 derives from a homologous recombination between 2 nearly identical 1041–base pair (bp) sequences, int1h-1 and int1h-2, in opposite orientation, positioned respectively in intron 1 of the gene and in a more telomeric region, 140 kilobases (kb) downstream, between the C6. 1A and VBPI genes. This rearrangement results in the production of 2 chimeric mRNAs; the first, possibly under the control of the F8 gene promoter, contains the first exon of the F8 gene, followed by some exons of VBP1. The other chimeric mRNA, under control of the C6. 1A …

El tratamiento medico de la EMB se basa fundamentalmente en la administracion de compuestos que contengan selenio y/o vitamina E, normalmente en la forma de selenito sodico y acetato de alfa-tocoferol. La aparicion de un brote de EMB en una explotacion de ovino extensivo de la comarca de Trujillo (Caceres), permitio la evaluacion de la eficacia de este tratamiento, mediante la determinacion de la actividad de la enzima glutacion peroxidasa (GSH-Px) en sangre entera y el seguimiento clinico de los animales. Los resultados obtenidos muestran los adecuados niveles adquiridos tras la administracion intramuscular se selenito sodico a las dosis recomendadas.