Acute liver failure as the first manifestation of very late relapsing of Hodgkin's disease

Hodgkin's disease is, in general, a lymph node-based disease. It usually starts in an area within the lymphatic system and spreads, in an orderly manner, along the lymphatic chain to contiguous lymph node areas. There have been sporadic case reports of acute liver failure caused by hematological malignancies. Generally, liver failure is a feature of stage IV end-stage disease, when it occurs in lymphoma. Thus, hepatic involvement usually occurs late in the course of Hodgkin's disease or with advanced-stage disease, and primary presentation in the liver with acute liver failure is extremely rare. In most cases, the diagnosis was made at autopsy. We describe a patient with Hodgkin's disease presenting with acute liver failure. This is a very unusual Hodgkin's disease form of presentation, because the acute liver failure was the presenting feature of the disease. Furthermore, the lymphoma occurred as a very late relapse, twenty years after the first diagnosis. To the best of our knowledge, such a case has not been described to date

Analysis of Intratumoral Heterogeneity in Myelodysplastic Syndromes with Isolated del(5q) Using a Single Cell Approach

Myelodysplastic syndromes (MDS) are a heterogeneous group of hematological diseases. Among them, the most well characterized subtype is MDS with isolated chromosome 5q deletion (MDS del(5q)), which is the only one defined by a cytogenetic abnormality that makes these patients candidates to be treated with lenalidomide. During the last decade, single cell (SC) analysis has emerged as a powerful tool to decipher clonal architecture and to further understand cancer and other diseases at higher resolution level compared to bulk sequencing techniques. In this study, a SC approach was used to analyze intratumoral heterogeneity in four patients with MDS del(5q). Single CD34+CD117+CD45+CD19- bone marrow hematopoietic stem progenitor cells were isolated using the C1 system (Fluidigm) from diagnosis or before receiving any treatment and from available follow-up samples. Selected somatic alterations were further analyzed in SC by high-throughput qPCR (Biomark HD, Fluidigm) using specific TaqMan assays. A median of 175 cells per sample were analyzed. Inferred clonal architectures were relatively simple and either linear or branching. Similar to previous studies based on bulk sequencing to infer clonal architecture, we were able to observe that an ancestral event in one patient can appear as a secondary hit in another one, thus reflecting the high intratumoral heterogeneity in MDS del(5q) and the importance of patient-specific molecular characterization

Fernando Huarte Morton (1921-2011): libro homenaje

La figura de Fernando Huarte Morton (1921-2011) tiene un gran significado para el mundo de las bibliotecas, el libro antiguo y la bibliografía española y goza de un profundo respeto, simpatía y admiración para todos aquellos que le conocieron y trataron personalmente. Desarrolló la mayor parte de su carrera profesional en la Biblioteca de la Universidad Complutense de Madrid, de la que fue director entre 1975 y 1986, contribuyendo a su modernización desde varios ámbitos: el reconocimiento institucional; la profesionalización del personal; la centralización de los fondos; la normalización de los procesos y servicios; el inicio de la mecanización del catálogo y la preocupación por el fondo antiguo y por la existencia de un edificio para albergar la gran biblioteca general. Con estas acciones, las bases de la biblioteca del siglo XXI estaban puestas. Es por este motivo, que la Biblioteca de la Universidad Complutense, institución a la que dedicó tantos años de su vida, quiera rendirle un sentido homenaje a través de la publicación de este libro en el que participan muchos de los bibliotecarios que le conocieron y que han querido dar a conocer su profesionalidad como bibliotecario, su rigor científico como estudioso e investigador y su atrayente personalidad llena de sentido del humor y de ironía

ICO-ICS Praxis para el tratamiento de la leucemia linfática crónica

Tractament mèdic; Tractament amb irradiació; Leucèmia limfàtica crònicaMedical treatment; Irradiation treatment; Chronic lymphocytic leukemiaTratamiento médico; Tratamiento con irradiación; Leucemia linfática crónicaLa leucèmia limfàtica crònica (LLC) és una alteració hematopoètica monoclonal caracteritzada per una expansió progressiva de limfòcits de la línia B. Aquests limfòcits, madurs des del punt de vista morfològic, però menys madurs des del punt de vista immunològic, s’acumulen a la sang, la medul·la òssia, els nòduls limfàtics i la melsa. Els principals objectius d’aquesta ICO-ICSPraxi són: - Desenvolupar, difondre, implementar i avaluar resultats de la ICO-ICSPraxi de la leucèmia limfàticacrònica (LLC). - Disminuir la variabilitat terapèutica entre els pacients tractats als diferents centres d'aquesta xarxa. - Implementar els resultats de la terapèutica en els pacients amb LLC tractats d'acord amb lesrecomanacions d'aquesta guia

ICO-ICS Praxis para el tratamiento médico y con irradiación del linfoma B difuso de célula grande

Tractament mèdic; Tractament amb irradiació; Limfoma B difús de cèl·lula granMedical treatment; Irradiation treatment; Diffuse large cell B lymphomaTratamiento médico; Tratamiento con irradiación; Linfoma B difuso de célula grandeEls limfomes no hodgkinians (LNH) són neoplàsies originades en cèl·lules limfoides en diferents estats maduratius, la qual cosa explica la gran heterogeneïtat biològica i clínica d’aquests tumors. Hi ha diversos sistemes de classificació, però el més utilitzat és la Classificació euroamericana revisada de limfomes de l'Organització Mundial de la Salut. El limfoma B difús de cèl·lula gran suposa aproximadament entre el 30-40% dels limfomes dels adults. Habitualment afecta adults amb una edat mitjana superior a 60 anys i el 60% dels pacients presenten els anomenats símptomes B (febre de 38 ºC o més, pèrdua de pes de més del 10% i/o sudoració nocturna. La majoria de casos són formes de novo però també poden ser deguts a la progressió o transformació d’una malaltia limfoproliferativa prèvia. Els objectius d'aquest document són: Desenvolupar, difondre, implementar i avaluar resultats de la ICO-ICSPraxi del limfoma B difús de cèl·lula gran. - Disminuir la variabilitat terapèutica entre els pacients tractats als diferents centres d'aquesta institució. - Implementar els resultats de la terapèutica en els pacients amb limfoma B difús de cèl·lula gran tractats d’acord amb les recomanacions d’aquesta guia

Pro-adrenomedullin usefulness in the management of children with community-acquired pneumonia, a preliminary prospective observational study

BackgroundIn adult population with community acquired pneumonia high levels of pro-adrenomedullin (pro-ADM) have been shown to be predictors of worse prognosis. The role of this biomarker in pediatric patients had not been analyzed to date. The objective of this study is to know the levels of pro-ADM in children with community acquired pneumonia (CAP) and analyze the relation between these levels and the patients¿ prognosis.FindingsProspective observational study including patients attended in the emergency service (January to October 2009) admitted to hospital with CAP and no complications at admission. The values for pro-ADM were analyzed in relation to: need for oxygen therapy, duration of oxygen therapy, fever and antibiotic therapy, complications, admission to the intensive care unit, and length of hospital stay. Fifty patients were included. Ten presented complications (7 pleural effusion). The median level of pro-ADM was 1.0065¿nmol/L (range 0.3715 to 7.2840¿nmol/L). The patients presenting complications had higher levels of pro-ADM (2.3190 vs. 1.1758¿nmol/L, p¿=¿0.013). Specifically, the presence of pleural effusion was associated with higher levels of pro-ADM (2.9440 vs. 1.1373¿nmol/L, p¿<¿0.001).ConclusionsIn our sample of patients admitted to hospital with CAP, pro-ADM levels are related to the development of complications during hospitalization

Pro-adrenomedullin usefulness in the management of children with community-acquired pneumonia, a preliminary prospective observational study

BackgroundIn adult population with community acquired pneumonia high levels of pro-adrenomedullin (pro-ADM) have been shown to be predictors of worse prognosis. The role of this biomarker in pediatric patients had not been analyzed to date. The objective of this study is to know the levels of pro-ADM in children with community acquired pneumonia (CAP) and analyze the relation between these levels and the patients¿ prognosis.FindingsProspective observational study including patients attended in the emergency service (January to October 2009) admitted to hospital with CAP and no complications at admission. The values for pro-ADM were analyzed in relation to: need for oxygen therapy, duration of oxygen therapy, fever and antibiotic therapy, complications, admission to the intensive care unit, and length of hospital stay. Fifty patients were included. Ten presented complications (7 pleural effusion). The median level of pro-ADM was 1.0065¿nmol/L (range 0.3715 to 7.2840¿nmol/L). The patients presenting complications had higher levels of pro-ADM (2.3190 vs. 1.1758¿nmol/L, p¿=¿0.013). Specifically, the presence of pleural effusion was associated with higher levels of pro-ADM (2.9440 vs. 1.1373¿nmol/L, p¿<¿0.001).ConclusionsIn our sample of patients admitted to hospital with CAP, pro-ADM levels are related to the development of complications during hospitalization

Analysis of Intratumoral Heterogeneity in Myelodysplastic Syndromes with Isolated del(5q) Using a Single Cell Approach

Myelodysplastic syndromes (MDS) are a heterogeneous group of hematological diseases. Among them, the most well characterized subtype is MDS with isolated chromosome 5q deletion (MDS del(5q)), which is the only one defined by a cytogenetic abnormality that makes these patients candidates to be treated with lenalidomide. During the last decade, single cell (SC) analysis has emerged as a powerful tool to decipher clonal architecture and to further understand cancer and other diseases at higher resolution level compared to bulk sequencing techniques. In this study, a SC approach was used to analyze intratumoral heterogeneity in four patients with MDS del(5q). Single CD34+CD117+CD45+CD19- bone marrow hematopoietic stem progenitor cells were isolated using the C1 system (Fluidigm) from diagnosis or before receiving any treatment and from available follow-up samples. Selected somatic alterations were further analyzed in SC by high-throughput qPCR (Biomark HD, Fluidigm) using specific TaqMan assays. A median of 175 cells per sample were analyzed. Inferred clonal architectures were relatively simple and either linear or branching. Similar to previous studies based on bulk sequencing to infer clonal architecture, we were able to observe that an ancestral event in one patient can appear as a secondary hit in another one, thus reflecting the high intratumoral heterogeneity in MDS del(5q) and the importance of patient-specific molecular characterization