Síndromes linfoproliferativos gastrointestinales / Gastrointestinal lymphoproliferative syndrome

The gastrointestinal lymphoid system plays a relevant role. The daily and continuous interaction between gastrointestinal lymphocytes with food and intestinal microbes requires precise functioning. The pathologic spectrum of lymphocyte malfunction results in lymphomas. MALT lymphoma is the most frequently diagnosed lymphoma, but there are other lymphoproliferative diseases such as diffuse large B cell lymphoma, mantle cell lymphoma and T associated lymphoma. The gastroenterologist and the endoscopist need to know these diseases in detail to achieve early diagnosis and treatment.

El sistema linfoide de defensa abdominal tiene un relevante rol en el buen funcionamiento sistémico. La interacción diaria y continua con patógenos alimentarios y microbios comensales intestinales precisa un estrecho funcionamiento. Las alteraciones linfoides clonales favorecen el desarrollo de linfomas de diversos tipos. Si bien, el linfoma asociado a tejido linfoide de mucosas (MALT) es el más conocido en contexto de su asociación con Helicobacter pylori, el tracto gastrointestinal se puede ver afectado por otros linfomas como el linfoma difuso de células grandes B y linfomas indolentes como el linfoma folicular, el linfoma del manto y el linfoma T asociado a enteropatía. El gastroenterólogo y endoscopista precisan conocer en detalle estas entidades para un oportuno diagnóstico y adecuado tratamiento.

Leucemia linfoblástica aguda con grave alteración hepática como manifestación inicial: presentación de dos casos y revisión de la literatura / Acute lymphoblastic leukemia causing hepatic failure: report of two cases

Hematological diseases may cause acute hepatic failure and are seldom sought. We report two cases illustrating this situation. A 16-year-old male presented with an acute liver failure: a bone marrow biopsy showed complete infiltration with lymphoid type blasts. A flow cytometry confirmed the presence of an acute lymphoblastic leukemia. The patient died days later. A 20-year-old female presented with acute liver failure: due to a pancytopenia, a bone marrow biopsy was performed, which confirmed the presence of an acute lymphoblastic leukemia. Chemotherapy was started immediately. The patient had a favorable evolution.

Ajuste de intensidad para el trasplante hematopoyético alogénico en leucemia aguda / Intensity adjustment of hematopoietic alogeneic transplantation in acute leukemia

Background: The intensity of conditioning chemotherapy and radiotherapy in hematopoietic stem cell transplantation (HSCT) varies according to several factors including the patient’s age, pre-existing conditions and performance status. Myeloablative conditioning (MA) increases transplant related mortality and reduces survival in older patients. Reduced intensity conditioning (RIC) is a good option for these patients. Aim: To report our experience with HSCT in patients of different ages with acute leukemia. Material and Methods: Retrospective analysis of 115 allogeneic HSCT performed in patients with acute myeloid or lymphoblastic leukemia. Results: We analyzed the cohort of patients in groups according to age at transplantation: younger than 40 years (n = 74), 41 to 50 years (n = 25) and older than 51 years of age (n = 16). Overall survival (OS), Disease free survival (DFS) and relapse at five years were similar in both groups of patients younger than 50 years (OS 40 and 44% respectively, DFS 38 and 42% respectively and relapse 40% and 34% respectively, p = NS). Patients over 51 years had a five years OS of 12%. However when we analyzed those patients by date and conditioning we found that patients who were treated with MA regimens in the first decade of the transplant program (before 2000) had lower OS compared to those treated after 2000 with RIC (five years OS 49% and 12% respectively, p < 0.01). No significant differences in terms of OS, recurrence or incidence of graft-versus-host disease were found when comparing groups under 40 years, between 41 and 50 years and older than 51 years treated only with RIC. Conclusions: RIC provides the possibility of HSCT in older patients with rates comparable to those obtained in younger patients successfully treated with MA conditioning.

[Intensity adjustment of hematopoietic alogeneic transplantation in acute leukemia]. / Ajuste de intensidad para el trasplante hematopoyético alogénico en leucemia aguda.

BACKGROUND: The intensity of conditioning chemotherapy and radiotherapy in hematopoietic stem cell transplantation (HSCT) varies according to several factors including the patient’s age, pre-existing conditions and performance status. Myeloablative conditioning (MA) increases transplant related mortality and reduces survival in older patients. Reduced intensity conditioning (RIC) is a good option for these patients. AIM: To report our experience with HSCT in patients of different ages with acute leukemia. MATERIAL AND METHODS: Retrospective analysis of 115 allogeneic HSCT performed in patients with acute myeloid or lymphoblastic leukemia. RESULTS: We analyzed the cohort of patients in groups according to age at transplantation: younger than 40 years (n = 74), 41 to 50 years (n = 25) and older than 51 years of age (n = 16). Overall survival (OS), Disease free survival (DFS) and relapse at five years were similar in both groups of patients younger than 50 years (OS 40 and 44% respectively, DFS 38 and 42% respectively and relapse 40% and 34% respectively, p = NS). Patients over 51 years had a five years OS of 12%. However when we analyzed those patients by date and conditioning we found that patients who were treated with MA regimens in the first decade of the transplant program (before 2000) had lower OS compared to those treated after 2000 with RIC (five years OS 49% and 12% respectively, p < 0.01). No significant differences in terms of OS, recurrence or incidence of graft-versus-host disease were found when comparing groups under 40 years, between 41 and 50 years and older than 51 years treated only with RIC. CONCLUSIONS: RIC provides the possibility of HSCT in older patients with rates comparable to those obtained in younger patients successfully treated with MA conditioning.

Amiloidosis por cadenas livianas de inmunoglobulina con compromiso gástrico al debut: revisión de la literatura basada en un caso clínico / Gastric AL amyloidosis as initial manifestation: literature review from a case report

Systemic amyloidosis comprises a group of diseases characterized by low molecular weight subunit protein deposit in organs, including the gastrointestinal tract. The most frequent clinical manifestations are gastrointestinal bleeding, malabsorption syndrome, protein-losing enteropathy and chronic intestinal dysmotility. The diagnosis is confirmed with gastrointestinal tissue biopsy positive to Congo red stain or recognizing the amyloid fibrils by electron microscopy. The treatment is based in the management of gastrointestinal symptoms and chemotherapeutic drugs, including melphalan and prednisone or cyclophosphamide, bortezomib and prednisone. The bone marrow transplant is reserved for 70-year-old patients or younger without advanced comorbidities. We present a case of a patient with weight loss, anorexia, nausea and early satiety.

La amiloidosis sistémica a un conjunto de enfermedades caracterizadas por el depósito de subunidades fibrilares proteicas de bajo peso molecular en órganos, incluyendo el sistema digestivo. Sus manifestaciones clínicas más frecuentes son la hemorragia digestiva, síndrome malabsortivo, gastro-enteropatía perdedora de proteínas y dismotilidad gastrointestinal crónica. El diagnóstico se confirma con una biopsia de tejido gastrointestinal positiva a tinción rojo Congo o la visualización de fibrillas de amiloide mediante microscopia electrónica. El tratamiento está basado el manejo de los síntomas gastrointestinales y el oncológico, donde destacan esquemas quimioterapéuticos que incluyen melfalan y prednisona o ciclofosfamida, bortezomib y prednisona. El trasplante de médula ósea está reservado a pacientes menores de 70 años sin comorbilidades avanzadas. Presentamos en este artículo el caso de un paciente con baja de peso, anorexia, náuseas y saciedad precoz.

[Acquired hemophagocytic syndrome treated with HLH 94-04 chemotherapy protocol: Report of four cases]. / Síndrome hemofagocítico adquirido: reporte de casos de cuatro pacientes adultos tratados con protocolo HLH 94-04 y revisión de la literatura.

Hemophagocytic syndrome is a severe condition of excessive immune activation that has a high mortality in the absence of treatment. The syndrome is classified as primary if associated with congenital or hereditary problems, or secondary/acquired if associated with infectious, autoimmune or oncology diseases. We report four adult cases of the syndrome, one with viral, two with autoimmune and one with idiopathic causes who were successfully treated with HLH 94-04 chemotherapy protocol. Our experience shows that a high index of suspicion, early diagnosis and an opportune therapy are essential in the treatment of this disease.

Síndrome hemofagocítico adquirido: reporte de casos de cuatro pacientes adultos tratados con protocolo HLH 94-04 y revisión de la literatura / Acquired hemophagocytic syndrome treated with HLH 94-04 chemotherapy protocol: Report of four cases

Hemophagocytic syndrome is a severe condition of excessive immune activation that has a high mortality in the absence of treatment. The syndrome is classified as primary if associated with congenital or hereditary problems, or secondary/acquired if associated with infectious, autoimmune or oncology diseases. We report four adult cases of the syndrome, one with viral, two with autoimmune and one with idiopathic causes who were successfully treated with HLH 94-04 chemotherapy protocol. Our experience shows that a high index of suspicion, early diagnosis and an opportune therapy are essential in the treatment of this disease.

Características clínicas y ecocardiográficas de los derrames pericárdicos en pacientes del Hospital Universitario San Vicente de Paúl / Clinical and echocardiographic characteristics of pericardial effusions in patients at the San Vicente de Paúl University Hospital

Objetivos: determinar las características clínicas y ecocardiográficas de los derrames pericárdicos.Metodología: revisar retrospectivamente las historias clínicas de los pacientes con diagnóstico de derrame pericárdico hecho por ecocardiografía transtorácica entre febrero de 2000 y agosto de 2001.Resultados: se evaluaron las historias clínicas de 62 pacientes con diagnóstico de derrame pericárdico; 28 (45.2 por ciento) presentaron derrames leves, 19 (30.6 por ciento) derrames moderados y 15 (24.2 por ciento) derrames severos; 21 (33.9 por ciento) pacientes presentaron taponamiento cardíaco y el 50 por ciento de éstos tenían derrame severo. Se buscó la presencia de signos inflamatorios y se encontró que 14 (22.6 por ciento) de los pacientes los presentaban. Además, estos pacientes tuvieron 5 veces más probabilidad de presentar taponamiento cardíaco (OR = 5.40). La principal causa de derrames pericárdicos fue la uremia (22.6 por ciento), seguida por las neoplasias (14.5 por ciento), la pericarditis bacteriana (11.3 por ciento) y la pericarditis aguda idiopática (11.3 por ciento), otras causan produjeron el 40.3 por ciento restante.Conclusiones: en la mayoría de los pacientes la enfermedad de base ocasiona el derrame pericárdico. Cuando no hay una enfermedad de base aparente pueden utilizarse el tamaño del derrame, los signos inflamatorios y la presencia de taponamiento cardíaco para establecer la posible causa del derrame.