Morbidity and mortality associated with performing bone marrow aspiration and biopsy

Background: Bone marrow aspiration and biopsy are diagnostic methods in the study of hematological diseases. Complications are rare. Knowing the morbidity and mortality related to the procedure is essential in order to implement preventive behaviors and improvement plans. Objective: To determine the incidence of complications in patients over 18 years who were undergone to bone marrow biopsy and aspiration in a university hospital between October 2013 and May of 2015. Furthermore, frequency, socio-demographic and clinical characteristics were established as well. Materials & methods: Observational, descriptive and retrospective study. The unit of analysis was biopsies and bone marrow aspirations. The information was obtained from the database of outpatient hematology ward, into the program Group of Education and Monitoring of Egress (GESE) of the Hematology Service of Hospital de San José (Bogotá, Colombia), and from medical records. Results: A total of 1252 bone marrow aspirates and biopsies were performed on 914 patients. Seventy-seven complications were reported, which corresponds to 6.15% of all documented procedures. The most frequent complication was: pain (100%), being more affected the women (66%), OR 1,91(IC 1,18-3,11) p=0,003. Regard to pathology diagnosis, 53.2% of biopsies were histologically normal hematopoiesis, followed by 16.8% for chronic myeloproliferative syndromes, which was the diagnosis most commonly associated with bleeding events (40%):OR 8,9 (IC 1,2-66,44) p=0,006, and death (1.3%). Conclusion: Pain was the most frequently reported complication, and among bleeding complications, chronic myeloproliferative disorders were the most common diagnosis. The largest number of complications in women may be related to the anatomical differences between the genders. It is recommended to improve post-procedure analgesia and prospective studies to establish association between complications and diagnosis

Niemann-Pick Disease: An Approach for Diagnosis in Adulthood

Niemann-Pick (NP) disease is a rare, autosomal recessive disorder characterized by visceromegaly and neurological alterations due to the excessive storage of lipids, sphingomyelin, and cholesterol. It commonly affects the child population, and only 6% of it occurs in the adult population. Type A is classified as the acute form, type B is the latest and with the best prognosis, and type C is characterized by neurological alteration. The diagnosis is based on enzymatic tests and genetic sequencing, with the latter being the diagnostic confirmation test. No specific treatment exists for this entity, although some patients with NPC type C may benefit from pharmacological treatment with miglustat. The objective of this paper is to describe the clinical characteristics of a grown patient with Niemann-Pick diagnosis type B. This article reports the case of a 55-year-old adult patient with a three-year clinical history consisting of splenomegaly and hematological disorders, without neurological symptoms ruling out frequent pathologies. Type B NP disease is diagnosed by a mutation in the sphingomyelin phosphodiesterase 1 (SMPD1) gene. The patient was receiving multidisciplinary support treatment. Although NP disease is a rare disease according to the literature, it is important to consider this group of disorders as a differential diagnosis, when other more common pathologies have been ruled out in patients with isolated splenomegaly and thrombocytopeni

Clinical and paraclinical characteristics of chronic myeloproliferative neoplasms Philadelphia chromosome negative

Objetivo: describir las características clínicas y paraclínicas de los pacientes con neoplasias mielo-proliferativas crónicas cromosoma Filadelfia negativa valorados en la consulta externa de hematología del Hospital de San José desde enero de 2005 hasta mayo de 2010.Material y métodos: estudio de serie de casos en el que se incluyeron los pacientes diagnosticados con neoplasias mieloproliferativas crónicas cromosoma Filadelfia negativas.Resultados: un total de 34 pacientes con neoplasias mieloproliferativas (NM) cromosomas Filadelfia negativas fueron identificados. El principal diagnóstico encontrado fue de trombocitemia esencial en 17 pacientes (50%), policitemia Vera Rubra en seis pacientes (17.6%), neoplasia mieloproliferativa asociadas a eosinofilia en seis pacientes (17.6%), mielofibrosis primaria en tres pacientes (8.8%) Y neoplasias mieloproliferativas no clasificables en dos pacientes (5.8%). La mediana de edad fue de 63.5 años (RIQ: 51 a 74) y 21 pacientes (61.7%) correspondían al sexo femenino. Dos pacientes del número total progresaron a mielofibrosis (5.8%), ningún paciente desarrolló leucemia aguda. Veintisiete pacientes (79.4%) recibieron hidroxiurea como manejo farmacológico principal. Catorce pacientes presentaron complicaciones (41.1%), de los cuales cinco fueron episodios trombóticos (14.7%), tres episodios hemorrágicos (8.8%), tres pacientes presentaron hipertensión pulmonar (8.8%) y un paciente desarrolló vértigo (2.9%). Finalmente el tiempo desde el diagnóstico hasta la aparición de complicaciones fue de 19.55 meses (RIQ: 8-50.23)Conclusiones: las neoplasias mieloproliferativas crónicas cromosoma Filadelfia negativas son patologías muy raras, el mayor número se agrupan en trombocitemia esencial, policitemia Vera y neoplasias asociadas a eosinofilia. La principal opción terapéutica es la hidroxiurea con una baja toxi-cidad. No es posible analizar la presencia de las mutaciones tirosina-kinasas (JAK2 V617F, PGDFRA, PDGFRB y FGFR1) ya que son herramientas de reciente ingreso al arsenal diagnóstico y cuyo impacto como factor pronóstico o terapéutico se encuentra en estudio. Las complicaciones más frecuentemente encontradas en esta serie son los eventos trombóticos venosos.Objective: describe features clinics and patients with chronic myeloproliferative neoplasm chromosome Philadelphia negative valued at the Hospital San Jose hematology outpatient from January 2005 until May 2010. Materials and methods: Studio series case included patients diagnosed with chronic myeloproliferative neoplasm chromosome Philadelphia negative. Results: A total of 34 chronic myeloproliferative neoplasm chromosome Philadelphia negative patients were identified. The main found diagnosis was Essential Thrombocythemia in 17 patients (50%), Polycythemia Rubra Vera in six patients (17.6%), chronic myeloproliferative neoplasm associated with eosinophilia in six patients (17.6%), myelofibrosis primary in three patients (8.8%) and chronic myeloproliferative neoplasm not classifiable in two patients (5.8%). The median age was 63.5 years (R: 51-74) and 21 patients (61.7%) were female. Two patients in the total number progressed Myelofibrosis (5.8%), no patient acute leukemia development. Twenty-seven patients (79.4%) received Hydroxyurea as main pharmacological management. Fourteen patients presented complications (41.1%), of which five were thrombotic episodes (14.7%), three bleeding episodes (8.8%), three patients had pulmonary hypertension (8.8%) and one patient developed Vertigo (2.9%). Finally the time since diagnosis until the occurrence of complications was 19.55 months (R: 8-50.23). Conclusión: neoplasm Mieloproliferativas Chronicles are very rare pathologies, as many are grouped into essential Thrombocythemia, Polycythemia Vera and neoplasms associated with Eosinophilia. The main therapeutic option is with a low toxicity Hydroxyurea. It is not possible to analyze the presence of mutations tyrosine kinases (JAK2 V617F PGDFRA PDGFRB, FGFR1) because they are tools of recent entry to the diagnostic arsenal and whose impact as a prognostic factor or therapeutic is in studio. Thrombotic venous events are frequently found in these patients

Hematology-dental integrated management in Glanzmann thrombasthenia

La trombastenia de Glanzmann (TG) es una enfermedad hematológica de baja frecuencia, tiene un patrón de herencia autosómica recesiva. Se caracteriza por alteración de la función plaquetaria. Se puede presentar como petequias, epistaxis, hemorragias gastrointestinales y gingivales. Se reporta el caso de un paciente de género masculino que acude a servicio odontológico universitario anexo a hospital de cuarto nivel por presentar gingivorragia severa y aflojamiento de algunos dientes. El manejo integral del paciente muestra la importancia del trabajo multidisciplinario.Glanzmann’s thrombasthenia (GT) is a low frequency hematologic disease with an autosomal recessive inheritance pattern. It is characterized by impaired platelet function. It It can occur as petechiae, epistaxis and gastrointestinal and gingival bleeding. The case of a male patient who attends University Dental Service annex to fourth level hospital for presenting severe gingival bleeding and loosening of some teeth is reported. The integral management of the patient shows the importance of multidisciplinary work

Surgical management for ankle arthropathy in patients with hemophilia and other congenital coagulation disorders

Objective: Compare the surgical treatment options available in our institution and the postoperative outcomes during follow-up. Additionally, assess patient’s perception of their condition and its progression. Methods: This descriptive observational study is a case series with a narrative literature review. All patients who met the inclusion and exclusion criteria and received surgical management for ankle arthropathy due to ankle hemarthrosis between 1999 and 2022 at Hospital San José in Bogotá were included. A perception survey was conducted via teleconsultation to evaluate pain control, functional impact, and capacity for daily activities. Results: Fifteen patients were included in the study, 13 male (86.7%). The mean age at the time of surgical intervention was 33 years (range 25–68). Among the patients, eight (53.3%) were diagnosed with Hemophilia A, four (26.7%) with Hemophilia B, two with Von Willebrand disease (13.3%), and one (6.7%) with Factor VII deficiency. The most common surgical intervention was ankle arthroscopy, with nine cases (50%). No infectious or intraoperative complications were documented, but one case of Hemophilic Pseudotumor developed late. The mean follow-up time was 87.7 months (IQR 60–105, SD 56.17). All patients reported improved pain and resumed their daily activities after the surgical intervention. Conclusion: Surgical management, regardless of the technique used (joint preservation or sacrifice), had a positive impact on patients by reducing pain after conservative management failure and allowing them to regain their daily activities, improving their quality of life. Level of Evidence IV; Therapeutic studies - investigating the results of treatment; Case series

Calidad físico-química y microbiológica del agua en parques acuáticos

El propósito de esta investigación fue analizar la evolución de la calidad del agua en un parque acuático determinando niveles de deterioro y causas probables; para el estudio se analizaron diferentes parámetros determinando presencia de microorganismos con el fin de proponer sistemas de control de calidad del agua para estos establecimientos. Como zona de estudio se eligió el estado de Morelos, México, el cual cuenta con cuatro parques acuáticos. La investigación analizó uno de estos parques, dividiéndose así: Fase 1) Muestreo del agua en la entrada y salida del parque, analizando parámetros fisicoquímicos y microbiológicos; y Fase 2) Evaluación operativa en seis piscinas del parque acuático, valorándose la conductividad eléctrica, sólidos disueltos totales (SDT), pH, color, turbiedad y cloro residual. Los resultados fueron analizados con Normas Oficiales Mexicanas para determinar si existe o no deterioro en la calidad del recurso en procesos operativos del parque. La Fase 1 dio como resultados elevados niveles en grasas y aceites (19.0 mg/L), color (100 UPt-Co), coliformes fecales (1.15.104 NMP 100 mL) y coliformes totales (1.70.105 NMP/100 mL). La Fase 2 mostró elevados valores de pH (7.9-8.4), disminuyendo eficiencia en la desinfección y comportamiento diferenciado en conductividad y SDT en piscinas con sistemas de filtrado. Como conclusiones se determinó que la calidad del agua en parques acuáticos debe controlarse en paralelo con la operatividad en piscinas, los procesos de cloración deben estandarizarse, que los usuarios son responsables del deterioro y que es necesaria la generación de un Sistema de Gestión Ambiental

Prophylaxis with activated prothrombin complex concentrate in patients with hemophilia A with high response inhibitors .An alternative approach to standard treatment

Introducción: la complicación más grave de los pacientes con hemofilia es el desarrollo de anticuerpos inhibidores; hasta un 30% de los pacientes con hemofilia A severa los desarrollan. Para erradicarlos, la inducción de tolerancia inmune es el tratamiento de elección; cuando persisten, los tratamientos profilácticos con agentes de puente como el concentrado de complejo de protrombina activado CCPa (FEIBA®) o rFVIIa (Novoseven®) ofrecen una alternativa terapéutica para reducir los sangrados y la artropatía hemofílica. Para evaluar la eficacia de profilaxis con CCPa se compararon los sangrados antes y después de recibir profilaxis (11-12 meses) en ocho pacientes hemofílicos con inhibidores de alta respuesta. Material y métodos: se realizó un estudio multicéntrico, se incluyeron niños y adultos con diagnóstico de hemofilia A, con título de inhibidores altos, de cuatro centros de atención en dos ciudades. Se excluyeron pacientes con hemofilia adquirida. Resultados: seis pacientes tenían hemofilia A severa y dos moderada; 7/8 pacientes tenían artropatía hemofílica. La media de edad fue 19 años (rango 7-38) y la del título de inhibidor 80 UB (rango 15-1178). La dosis de CCPa fluctuó entre 40 y 75 U/kg, dos a tres veces por semana. Las tasas anuales de sangrado global y de hemartrosis previas a profilaxis fueron (8/año y 3.1/año) y después de profilaxis durante un periodo de 11-12 meses fueron (1.08/año y 1/año); se encontró una reducción de 86 y 68% respectivamente. No hubo eventos de trombosis. El cumplimiento del esquema de tratamiento con CCPa fue mayor a 80%. Conclusiones: este es el primer reporte de casos en Colombia sobre el uso de CCPa en pacientes hemofílicos con inhibidores del factor VIII de alta respuesta. Persisten interrogantes sobre la duración o ajustes al esquema de tratamiento.The most serious complication of hemophilia patients is the development of inhibitory antibodies; up to 30% of patients with severe hemophilia A develop them. To eradicate these antibodies, induction of immune tolerance is the treatment of choice; when they persist, prophylactic treatment with bridge agents as activated prothrombin complex concentrate aPCC (FEIBA®) or rFVIIa (Novoseven®) offer a therapeutic alternative for reducing bleeding and hemophilic arthropathy. To evaluate the efficacy of prophylaxis with aPCC, bleeds were compared before and after receiving prophylaxis (11-12 months) in 8 hemophilia patients with high response inhibitors. Material and methods: a multicenter study was conducted in children and adults with a diagnosis of hemophilia A with high titer inhibitors in 4 attention centers in two cities. Patients with acquired haemophilia were excluded. Results: six patients had severe hemophilia A and 2 moderate; 7/8 patients had hemophilic arthropathy. The mean age was 19 years (range 7-38) and mean inhibitor titer was 80 UB (range 15- 1178). aPCC dose ranged from 40-75 U / kg, 2-3 times a week. The overall annual rates of bleeding and hemarthrosis pre-prophylaxis were (8 / year and 3.1 / yr) and after prophylaxis during a period of 11- 12 months were (1.08 / year and 1 / year); a reduction of 86% and 68% respectively was found. There were no thrombotic events. Compliance scheme of aPCC treatment was higher than 80%. Conclusions: this is the first case report in Colombia on the use of aPCC in haemophilia patients with high responding inhibitors to factor VIII. Questions remain about the length or adjustments to the treatment schedul

Complicaciones posteriores a procedimientos invasivos en los pacientes anticoagulados intervenidos en el Hospital de San José, Bogotá, Colombia

El manejo perioperatorio de los pacientes previamente anticoagulados es un problema muy común en la práctica clínica diaria. El manejo de estos pacientes es cambiante y depende mucho del personal médico que los intervenga, quienes establecen el riesgo entre las complicaciones trombóticas al suspender la terapia anticoagulante y el riesgo de sangrado antes y después del evento quirúrgico. Objetivo: Describir las características hematológicas y sus complicaciones trombóticas o hemorrágicas de los pacientes anticoagulados que son llevados a cirugías o procedimientos invasivos mayores o menores, bajo anestesia general, regional o local, en el Hospital San José durante el mes de septiembre y octubre de 2009. El propósito de este proyecto corresponde a la identificación de un manejo adecuado de los servicios quirúrgicos para disminuir morbi-mortalidad relacionadas con estas complicaciones. Método: El tipo de estudio es observacional descriptivo en una cohorte prospectiva de pacientes que fueron llevados a cirugía o procedimientos invasivos en el Hospital San José. El seguimiento de los pacientes fue hecho por un mes adicional después de realizado procedimiento para establecer las complicaciones trombóticas y hemorrágicas posteriores al cambio o suspensión de la anticoagulación. Resultados: De los 30 pacientes, 15 pacientes presentaron complicaciones; el 93.3% (14 pacientes) fueron de tipo hemorrágico y solo un paciente presentó una complicación trombótica. Las complicaciones hemorrágicas se presentaron en 11 pacientes que fueron sometidos a un procedimiento mayor, mientras solo 3 sometidos a procedimientos menores. Conclusiones: En el periodo postoperatorio del Hospital San José se documentó un aumento de los eventos hemorrágicos en el periodo post operatorio, en comparación con los eventos trombóticos. Adicionalmente se documentó una gran heterogeneidad en el manejo de la anticoagulación, con poca estratificación del riesgo de sangrado o trombosis, y del manejo usual de las complicaciones

Interpretação do hemograma automatizado através de um Objeto Virtual de Aprendizagem (OVA): Descrição da experiência

The virtual learning objects have generated positive changes in the teaching process, availability and accessibility to knowledge; unlike of the traditional learning model, it has as a purpose, to offer more flexible mechanisms and allow multiple queries to consolidate a broader content of knowledge. The complete blood cell count is a laboratory test of daily use in medicine, its interpretation demands a precise and solid understanding for the decision making. This article has the finality to describe the experience and the phases for the construction of an virtual learning object in hematology based in the ADDIE model, as follows: phase I: analysis, phase II: design, phase III: development, phase IV: implementation, phase V: evaluation. The setting-up of a virtual learning object demands the methodical accomplishment of process to assure the adjustment of the results to the requirements of the learning tools. The improvement in the understanding of the blood cell count has importance in the impact as a study element, as well as in the encouragement in the competences for make assertive decisions and the rationality in the request of diagnosis studies, however, to prove the effectiveness as a learning strategy a pilot test is required.Los Objetos Virtuales de Aprendizaje han generado cambios positivos en la enseñanza, disponibilidad y accesibilidad al conocimiento; a diferencia del modelo tradicional de aprendizaje, tienen como propósito ofrecer mecanismos más flexibles y de múltiple consulta para consolidar un contenido más amplio del conocimiento. El hemograma es un examen de laboratorio de uso diario en medicina; su interpretación exige un entendimiento preciso y sólido para la toma de decisiones. Este artículo describe la experiencia y las fases para construir un objeto virtual de aprendizaje en hematología, basado en el modelo ADDIE (Análisis, Diseño, Desarrollo, Implementación y Evaluación). Crear un objeto virtual de aprendizaje exige el cumplimiento metódico de procesos para garantizar que el resultado se ajuste a los requerimientos de las herramientas de aprendizaje. Mejorar el entendimiento del hemograma es importante en la formación y fomento de competencias para la toma de decisiones asertivas y la racionalidad en la solicitud de estudios diagnósticos; no obstante, para probar la efectividad del OVA como estrategia de aprendizaje se requiere de una prueba piloto.Os Objetos Virtuais de Aprendizagem (OVA) geraram mudanças positivas no ensino, disponibilidade e acessibilidade ao conhecimento; ao contrário do modelo de aprendizagem tradicional, eles visam oferecer mecanismos de consulta mais flexíveis e múltiplos para consolidar um conteúdo mais amplo de conhecimento. O hemograma completo é um teste laboratorial para uso diário em medicina; sua intepretação requer um entendimento preciso e solido para tomada de decisões. Este artigo descreve a experiência e as fases para construir um OVA em hematologia, com base no modelo ADDIE (Análise, Projeto, Desenvolvimento, Implementação e Avaliação). Criar um OVA requer conformidade metódica com processos para garantir que o resultado atenda aos requisitos das ferramentas de aprendizado. Melhorar a compreensão do hemograma é importante no treinamento e na promoção de habilidades para tomar decisões assertivas e racionalidade na solicitação de estudos de diagnósticos; no entanto, para testar e eficácia do OVA como estratégia de aprendizagem, é necessário um teste piloto

Musculoskeletal evaluation in severe haemophilia A patients from Latin America

There is a paucity of literature on haemophilia treatment in Latin American countries, a region characterized by rapidly improving systems of care, but with substantial disparities in treatment between countries. The aim of this study was to evaluate the musculoskeletal status of haemophilia patients from Latin America and to examine the relationship between musculoskeletal status and treatment practices across countries. The Committee of Latin America on the Therapeutics of Inhibitor Groups conducted a survey of its member country representatives on key aspects of haemophilia treatment in 10 countries. Musculoskeletal status of patients was obtained during routine comprehensive evaluations between March 2009 and March 2011. Eligible patients had severe haemophilia A (factor VIII <1%) without inhibitors (<0.6 BU mL−1) and were ≥5 years of age. Musculoskeletal status was compared between three groups of countries, based primarily on differences in the availability of long‐term prophylaxis. Overall, 143 patients (5–66 years of age) were enrolled from nine countries. In countries where long‐term prophylaxis had been available for at least 10 years (Group A), patients aged 5–10 years had significantly better mean World Federation of Hemophilia clinical scores, fewer target joints and fewer affected joints than patients from countries where long‐term prophylaxis has been available for about 5 years (Group B) or was not available (Group C). In Latin America, the musculoskeletal status of patients with severe haemophilia without inhibitors has improved significantly in association with the provision of long‐term prophylaxis. As more countries in Latin America institute this practice, further improvements are anticipated