There are previously reported data describing differences between Asian and European patients with Myelodysplastic Syndromes (MDS), few direct comparisons based on cancer registration characteristics or using cohorts to validate scoring systems. This is the first study from South-America, which attempts to describe demographic, clinical features, and outcome of MDS patients. We retrospectively analyzed 1,080 patients with de novo MDS from Argentina (635), Brazil (345), and Chile (100). Chilean patients were younger (P 5 0.001) with female preponderance (P 5 0.071). Brazilian series showed a higher predominance of RARS subtype regarding FAB and WHO classifications (P < 0.001). Hemoglobin levels were significantly lower in Brazilian and Chilean series (P < 0.001), and Chilean series also showed a lower platelet count (P 5 0.028), with no differences concerning the neutrophil count, % BM blast, and the distribution of cytogenetic risk groups (P > 0.05). Chilean series depicted a lower overall survival (OS; 35 months vs. 56 months-Argentine; 55 monthsBrazil, P 5 0.030), which was consistent with a higher predominance of the high-risk group according both to the IPSS and IPSS-R (P 5 0.046 and P < 0.001). The IPSS-R system and its variables showed a good reproducibility to predict clinical outcome for the whole South-American population. Epidemiological and clinical characteristics, distribution among prognostic subgroups, the OS, and the access to disease modifying therapies were more similar between Argentinean and Brazilian compared with Chilean MDS series. This will need further analysis in a larger group of patients. Descriptive and comparative studies are necessary to establish epidemiological features useful for public health attitudes to generate suitable therapeutic schemes.

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(Rev Med Chile 2017; 145: 812-815)

Background: Philadelphia-negative Myeloproliferative Neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are Polycythemia Vera (PV), Essential Thrombocytopenia (ET) and Myelofibrosis (MF). The diagnosis includes clinical, histological and molecular features. There are not data from Chile. The aim of this study is to determinate epidemiological, clinical, diagnostic and therapeutic characteristics of Ph-MPN in our country. Methods: Descriptive and retrospective study. We reviewed the database of the Molecular Biology Laboratory at the Hospital del Salvador, a national reference laboratory, from 2012 to 2017. All patients referred as Ph-MPN were included. We reviewed the clinical records to obtain clinical information. Results: Clinical data was obtained from 468 cases from 12 public hospitals in Chile. Median age at diagnosis was 70 years. Female to Male ratio= 1,15:1, without significant differences between Ph-MPNs. ET was the most frequently Ph-MNP found, accounting for 49,4% of all Ph-MPN, followed by PV (37%) and MF (10,4%). A 66,2% of ET was JAK2 V617F+. Bone marrow biopsy was performed in 35% of ET cases. Only 7,8% had cytogenetic study. Splenomegaly was found in 8%. Thrombosis was observed in 23,8%. The median platelet count was 842x109/L. All patients received hydrea +/- aspirin or oral anticoagulation. Of the total of PV, 86,6% was JAK2+. Bone marrow biopsy was performed in a quarter of the cases. Thrombosis frequency was 14,5%. A 29% had splenomegaly. Median hemoglobin level was 18 gr/dl. All patients were treated with aspirin +/- phlebotomy and about half of them required cytoreduction. Two patients were refractory to hydrea and used ruxolitinib as second line treatment. A 63,3% of the MF were JAK-2+. Bone marrow biopsy was performed in 59% and 20% had a cytogenetic study. Only one fifth of patients had LDH measurement at diagnosis. Splenomegaly was observed in 75,5% of cases. Thrombosis frequency was 13%. Anemia was the most frequent finding in complete blood count. The treatments were heterogeneous, including hydrea, EPO, thalidomide/prednisone, danazol and ruxolitinib. Discussion: TE was the most common Ph-MPN. The epidemiological and blood count findings were similar to the data reported in the literature. It is important to note that with the 2016 WHO classification new criteria, some of patients diagnosed with ET, now will be in PV cathegory (21 patients in our serie). The distribution of JAK2V617F+ in Ph-MPN was similar to the published data, except for PV, in which we found a lower percentage of JAK2+. Thrombosis were lower than the data reported for PV. It is worrisome that bone marrow biopsy and cytogenetic study were performed only in a low percentage of the patients. The treatment strategies were heterogeneous and not standardized among the participating centers. These findings reveal a lack in the use of the diagnostic tools for Ph-MPN. It is important to improve clinical and molecular characterization of these patients in order to guide available therapeutic alternatives in our country. Disclosures No relevant conflicts of interest to declare.

Liver transplantation in hemophilia A. Report of one case Due to blood derivative requirements, many patients with hemophilia were exposed to Hepatitis C virus infection (HCV) before the availability of HCV testing. We report a 46-year-old male with Hemophilia A with a hepatitis virus C infection since 2004 causing a cirrhosis. Due to a hepatopulmonary syndrome, he received a liver allograph using a factor VIII replacement protocol, after eradicating the virus C. He had a good postoperative evolution, and no more factor VIII was required after transplantation until his last assessment.

Acquired aplastic anemia. Experience in a public hospital Background: The first line treatment for patients < 40 years old with aplastic anemia (AA) is allogeneic HLA-identical sibling donor transplantation (SCT). Immunosuppressive therapy (IST) with a combination of Thymoglobuline (ATG) and cyclosporine is used for older patients or those without a donor. Five year overall survival (OS) for both therapies is > 70%. Aim: To report the experience with SCT and ATG for AA in a public hospital. Patients and Methods: AA was diagnosed in 42 patients between 1998 and 2016, according to Camitta criteria. Thirty eight (90%) received treatment, 7 (18%) under 40 years old received SCT, and 31 (82%) IST. The rest were not treated. OS was calculated from date of diagnosis until last control, death or loss from follow up. Results: Complete or partial hematologic response, was obtained in 71% and 58% of cases with SCT and IS, respectively. Five year OS was 71% and 55% with SCT and IST, respectively. No difference in response was observed between horse and rabbit ATG. Conclusions: SCT from an HLA-identical sibling donor had a high response rate and survival. IST instead, had a lower response and survival, due to an initial high mortality rate.

Background: Renal failure (RF) is a common complication in patients with newly diagnosed multiple myeloma (NDMM). Aim: To evaluate the frequency of RF in NDMM patients, and the prognostic impact of its reversibility. Material and Methods: A retrospective study evaluating demographic and clinical characteristics of 154 consecutive patients with NDMM was carried out. Estimated glomerular filtration rate (eGFR) was calculated at the beginning and at the end of the induction therapy. In addition, we evaluated renal responses (RR) according to the International Myeloma Working Group (IMWG) criteria. The induction regimen was based on thalidomide in all cases. Results: Fifty-three patients had RF (34.4%). Complete renal response (RR) was achieved in 51%. Three years overall survival in patients without RF, with RF and complete RR, and patients with RF and any other RR, was 66, 47 and 13%, respectively. Median survival was 53, 27 and 6 months, respectively (p < 0.01). In the multivariate analysis, RF and hypercalcemia were independent predictors of a worse outcome. Conclusions: Achieving a complete RR in patients with NDMM, is associated with a better survival. (Rev Med Chile 2019; 147: 1374-1381 Impacto pronóstico de remisión renal en pacientes con mieloma múltiple de reciente diagnóstico Antecedentes: La falla renal (FR) es una complicación frecuente en pacientes con mieloma múltiple (MM). Objetivo: Evaluar la frecuencia de FR en pacientes con reciente diagnóstico de MM y determinar la importancia pronóstica de su reversibilidad. Material y Métodos: Se realizó un estudio retrospectivo de 154 pacientes consecutivos con MM. La función renal se evaluó mediante la tasa estimada de filtración glomerular al inicio y final de la terapia de inducción. Además, evaluamos las respuestas renales (RR) de acuerdo con los criterios del International Myeloma Working Group (IMWG). El régimen de inducción se basó en talidomida en todos los casos. Resultados: Cincuenta y tres pacientes presentaron FR (34,4%) al diagnóstico. La RR completa se logró en 51%. La sobrevida global (SG) a 3 años en pacientes sin FR, con FR y RR completa, y