Aplicación del índice de mortalidad pediátrica 3 y evaluación de falla orgánica secuencial pediátrica en una unidad de cuidados intensivos en México / Implementation of the Pediatric Index of Mortality 3 and the pediatric Sequential Organ Failure Assessment in an intensive care unit in Mexico

Introducción. El objetivo del estudio fue analizar el índice de mortalidad pediátrica 3 (PIM 3) y la evaluación de falla orgánica secuencial pediátrica (pSOFA) para predicción de muerte. Métodos. Estudio observacional prospectivo; se incluyeron pacientes de 1 mes a 17,9 años. La precisión se evaluó con el área bajo la curva (AUC) y se estimó la tasa de mortalidad estandarizada. Resultados. Se estudiaron 244 ingresos; la mediana de edad fue 60 meses. Los diagnósticos principales fueron neoplasias sólidas o hematológicas (26,5 %). La mortalidad por ingresos fue del 18 % (44/244). Para PIM 3 el AUC fue de 0,77 y para pSOFA, de 0,81; ambas escalas mostraron adecuada calibración (p > 0,05). La tasa de mortalidad estandarizada fue de 1,91. Conclusiones. Identificamos que las escalas de evaluación de mortalidad PIM 3 y pSOFA muestran capacidad de discriminación aceptable. En pacientes con neoplasias sólidas o hematológicas, PIM 3 no mostró adecuada calibración.

Introduction. The study objective was to analyze the Pediatric Index of Mortality 3 (PIM 3) and the pediatric Sequential Organ Failure Assessment (pSOFA) for the prediction of mortality. Methods. Observational, prospective study; patients aged 1 month to 17.9 years were included. Assessment of area under the curve (AUC) accuracy and estimation of standardized mortality rate. Results. A total of 244 admissions were studied: median age was 60 months. The main diagnoses were solid or hematologic neoplasms (26.5%). The mortality by admission was 18% (44/244). The AUC was 0.77 for PIM 3 and 0.81 for pSOFA; both scales showed an adequate calibration (p > 0.05). The standardized mortality rate was 1.91. Conclusions. We identified that the PIM 3 and pSOFA have an acceptable discrimination power. The calibration of the PIM 3 was not adequate in patients with solid or hematologic neoplasms.

Resignificación de la vida por parte de jóvenes sobrevivientes al cáncer hematológico / Resignification of Life by Young People Survivors of Hematologic Cancer

Introducción: Los jóvenes con enfermedades oncológicas enfrentan dificultades durante los períodos de diagnóstico, tratamiento y recuperación. Estos procesos complejos cargados de experiencias vitales inusitadas producen la transformación de su conciencia y los lleva a construir nuevos significados en la manera de comprender, relacionarse y actuar en el mundo que los rodea. Objetivo: Comprender el (re)significado de la vida a partir de la experiencia de los jóvenes que sobrevivieron al cáncer hematológico. Métodos: Estudio cualitativo, que utilizó la Teoría Fundamentada en Datos como metodología y el referencial de la Teoría de la Complejidad de Morin. Se realizaron entrevistas en profundidad a 12 adolescentes sobrevivientes de cáncer hematológico. El tamaño de muestra fue determinado al alcanzar nivel de saturación. El análisis fue simultáneo durante la recolección de los datos, mediante codificación abierta, axial y selectiva según lo señalan Strauss y Corbin. Resultados: Emergieron dos categorías: Reorganizando su vida por medio de cambios y aprendizajes para vencer al cáncer y, Asumiendo una mejor comprensión y compromiso con los demás y consigo mismo. Conclusiones: Las experiencias vividas por jóvenes sobrevivientes que padecen de cáncer modifican su forma de vivir y se tornan más comprensivos con el sufrimiento que ocasiona la enfermedad. Esta situación los hace más solidarios y comprometidos con su contexto social sobre todo con su familia y con pacientes oncológicos(AU)

Introduction: Young people with oncological diseases face difficulties during the periods of diagnosis, treatment and recovery. These complex processes loaded with unusual life experiences produce the transformation of their consciousness and lead them to construct new meanings in the way that they understand, relate and act in the world around them. Objective: To understand the (re)signification of life from the experience of young survivors of hematological cancer. Methods: A qualitative study was carried out, using the data driven theory as the methodology and Morin's complexity theory as the referent. In-depth interviews were conducted with twelve adolescent hematologic cancer survivors. The sample size was determined by reaching the saturation level. The analysis was simultaneous during data collection, using open, axial and selective coding according to Strauss and Corbin. Results: Two categories emerged: 1. reorganizing their life through changes and learning to overcome cancer and 2. assuming a better understanding and commitment to others and to themselves. Conclusions: The experiences lived by young cancer survivors modify their way of living as they become more understanding of the suffering caused by the disease. This situation makes them more supportive and committed to their social context, especially with their family and with cancer patients(AU)

Características otorrinolaringológicas en niños con trisomía 21: un estudio de 171 pacientes en el Hospital Infantil de México Federico Gómez / Otolaryngological features in children with trisomy 21: a study of 171 patients at the Hospital Infantil de México Federico Gómez

Resumen Introducción: Los niños con trisomía 21 enfrentan una amplia gama de problemas en la región de la cabeza y el cuello, por lo cual es importante reconocer las manifestaciones otorrinolaringológicas que presentan, así como su apropiado manejo. Métodos: Estudio de serie de casos retrospectivo de pacientes pediátricos con trisomía 21. De cada caso se analizó el espectro de manifestaciones otorrinolaringológicas, el manejo establecido y los resultados. Resultados: Se incluyeron 171 niños. La edad media de la primera valoración por otorrinolaringología en la institución fue de 7.2 ± 4.2 años. Las manifestaciones otológicas más frecuentes fueron la estenosis del conducto auditivo externo y la disfunción de la trompa de Eustaquio. Más de la mitad de los pacientes (63 %) presentaron hipoacusia, principalmente de tipo conductivo bilateral, y hasta el 75 % de los pacientes con afectación otológica requirieron algún procedimiento quirúrgico. Las manifestaciones rinológicas más comunes fueron la rinosinusitis crónica y la rinitis alérgica. La apnea obstructiva del sueño estuvo presente en el 30% de los pacientes. El tratamiento principal fue la amigdalectomía, seguida del tratamiento con dispositivos de presión positiva de la vía aérea. Menos del 5 % de los pacientes presentaron un compromiso laríngeo. Conclusiones: Los pacientes pediátricos con trisomía 21 deben ser remitidos sistemáticamente a una evaluación otorrinolaringológica periódica, debido a la alta incidencia de manifestaciones en esta región. Se deben ofrecer tratamientos oportunos para mejorar su salud y calidad de vida.

Abstract Introduction: Children with trisomy 21 face a wide range of conditions in the head and neck region, for which it is important that physicians are aware and have a strong understanding of the ear, nose, and throat (ENT) disorders, and their management as well. Methods: Retrospective case series of pediatric patients with trisomy 21. The spectrum of otolaryngological manifestations, their management, and outcomes of each case were analysed. Results: One hundred and seventeen pediatric patients were included. The mean age was 7.2 ± 4.2 years. More than half of the patients (63 %) had hearing loss (HL). The most frequent presentation was conductive HL, predominating the mild and bilateral type. The most common otological manifestations found were external ear canal stenosis and Eustachian tube dysfunction. Up to 75 % of the patients with otologic involvement required some surgical procedure. The most common rhinological manifestations were chronic rhinosinusitis and allergic rhinitis. Obstructive sleep apnea (OSA) was present in 30% of all patients, which main treatment was tonsillectomy, followed by continuous positive and biphasic positive airway pressure treatments. Less than 5 % of the patients presented a laryngeal compromise. Conclusions: Pediatric patients with trisomy 21 systematically should be referred to periodic ENT assessment due to the high incidence of manifestations in this region. Timely treatments should be offered in order to improve the health and the quality of life of the patient.

Parte I. Requerimientos básicos de infectología para hospitales que atienden pacientes hemato-oncológicos: ambiente hospitalario, protocolos diagnósticos y arsenal terapéutico. Fundamentación / Basic requirements of facilities for hospitals that treat hemato-oncological patients: hospital environment, diagnostic protocols and therapeutic arsenal

Resumen La atención de pacientes con cáncer, incluyendo los receptores de trasplantes de precursores hematopoyéticos, plantea numerosos desafíos para los hospitales que deben proveer ambientes seguros, en que se logre aminorar al máximo posible la exposición a patógenos que generan morbilidad y mortalidad. Al mismo tiempo deben contar con protocolos establecidos que permitan realizar un estudio racional de las posibles etiologías infecciosas que pueden presentar estos pacientes. A su vez, deben asegurar la existencia de un arsenal terapéutico adecuado, junto a algoritmos de tratamiento oportuno, actualizado según guías consensuadas y efectivo según la infección sospechada o confirmada. En este artículo se introducen algunos de los argumentos que sustentan estos requerimientos que luego se desarrollan en tres artículos sucesivos dedicados al ambiente hospitalario, protocolos diagnósticos y arsenal terapéutico.

The care of cancer patients, including recipients of hematopoietic stem cell transplantation, has numerous challenges for hospitals that must provide safe environments in which exposure to pathogens that generate morbidity and mortality is reduced at maximum. At the same time, they must have established protocols that allow a rational study of the possible infectious etiologies and the existence of an adequate therapeutic arsenal together with timely treatment algorithms, updated according to consensus guidelines and effective according to the suspected or confirmed infection. This article introduces some of the arguments that support these requirements, then that are developed in three successive articles dedicated to the hospital environment, diagnostic protocols and therapeutic arsenal.

Reporte de la Primera Reunión Nacional de Consenso para la Inmunofenotipificación de Leucemias Agudas / Report of the First National Consensus Meeting for the Immunophenotyping of Acute Leukemias

Resumen En 2005 se publicaron recomendaciones para la tipificación de hemopatías malignas en Latinoamérica. Se consideró necesario realizar una reunión nacional para actualizarlas. Se convocaron y reunieron 95 profesionales expertos en el tema para analizar y contrastar alternativas y llegar a un consenso. Se alcanzaron opiniones de consenso en lo relativo a indicaciones, tipos y manejo de muestras, anticuerpos, nomenclatura e informe de resultados para el diagnóstico y seguimiento de las leucemias agudas. Las recomendaciones se describen en este artículo y se hace hincapié en la necesidad de que los laboratorios nacionales se apeguen a ellas.

Abstract Recommendations for the typing of hematological malignancies in Latin America were published in 2005. Carrying out a national meeting to update them was deemed necessary. 95 professional experts on the subject were invited in order to analyze and contrast alternatives and reach a consensus. Consensus opinions were reached regarding indications, sample types and processing, antibodies, nomenclature and reporting of results for the diagnosis and monitoring of acute leukemias. This paper describes the recommendations and emphasizes on the need for national laboratories to adhere to them.

Síndromes de hemopatías mieloides malignas hereditarias / Inherited myeloid malignancy syndromes

La predisposición de algunas familias a padecer hemopatías mieloides malignas ha sido descrita desde hace varias décadas; sin embargo, solo recientemente ha sido posible conocer las bases moleculares de estos síndromes. La importancia de reconocer y diagnosticar la presencia de mutaciones predisponentes de la línea germinal en pacientes con hemopatías malignas y en sus familiares determinó que la Organización Mundial de la Salud (OMS) introdujera esta nueva categoría en su última revisión de la clasificación de las neoplasias malignas y leucemias agudas. Mediante el uso de las modernas técnicas de biología molecular se ha logrado el descubrimiento de mutaciones en diferentes genes que aportan nuevos elementos en el proceso de leucemogénesis, permiten ofrecer consejo genético, una mejor selección del donante de médula ósea y se erigen en la fuente de futuras dianas terapéuticas. En este trabajo se revisan algunos de los síndromes de hemopatías mieloides malignas hereditarias (HMMH) y se enfatiza en la necesidad de realizar una exhaustiva historia clínica personal y familiar que permita un elevado índice de sospecha para el diagnóstico de estas entidades(AU)

The familial predisposition to inherited myeloid malignancies has been described since several decades ago; however, only recently have been possible to known the molecular basis of these syndromes. The importance to recognize and diagnosed predisposing germ line mutations in patients and relatives contributed to the introduction of this new category in the latest update of myeloid neoplasm and acute leukemia by World Health Organization (WHO). The use of modern molecular biology techniques has achieved the discovery of genetic mutations that shed light inside leukemogenesis process, allow offering a genetic counseling, a better donor selection and are the basis of future therapeutics targets. The main hereditary myeloid malignancy syndromes (IMMS) are reviewed, emphasizing the need of exhaustive personal and family clinical history and to have a high suspicion index to diagnose these entities(AU)

Hémopathies malignes à Brazzaville (Congo) : analyse de situation

L'épidémiologie des hémopathies malignes au Congo n'est pas connue. L'objectif de cette étude était de rapporter la distribution des hémopathies malignes à Brazzaville.Patients et méthodes : Il s'agissait d'une étude transversale descriptive réalisée dans le Service d'Hématologie Clinique du CHU de Brazzaville au Congo. La période étudiée est de 10 ans (du 1er janvier 2006 au 31 décembre 2015). Ont été inclus dans l'étude tous les dossiers de consultation et d'hospitalisation portant le diagnostic d'hémopathie maligne.Résultats : Deux cent-soixante-deux cas d'hémopathies malignes ont été diagnostiquées durant la période d'étude.Les hémopathies malignes ont été de typelymphoprolifératif chronique dans 57,3% (n = 150), leucémie aiguë dans 24, 4% (n = 64) et myéloprolifératif dans 18,3% (n = 48) des cas. Le myélome multiple et la leucémie aigüe lymphoblastique représentaient respectivement 29,8% (n = 70) et 19,1% (n = 50) des groupes nosologiques lymphoprolifératifs chroniques et leucémies aiguës. La leucémie myéloïde chronique représentait 100% du groupe myéloprolifératif. Une prédominance féminine a été observée (sex-ratio = 0,65). Les pathologies lymphoprolifératives chroniques intéressaient la tranche d'âge de 45 à 59 ans (66,7%), a leucémie myéloïde chronique celle de 15-29 ans (45,8%) et la leucémie aiguë lymphoblastique avait une distribution pédiatrique : 0-14 ans (68%).Conclusion : Les hémopathies malignes constituent par leur fréquence un problème sanitaire. Elles plaident pour des études épidémiologiques analytiques afin de mettre en place une politique préventive de celles-ci

Relações entre oncohematopediatras, mães e crianças na comunicação de notícias difíceis / Relationships between oncohematopediatrics, mothers and children in communicating bad news

Resumo Apresenta-se um estudo de relações entre oncohematopediatras, mães e crianças no compartilhamento de notícias difíceis (ND) num hospital público do Rio de Janeiro. O texto enfatiza o entrelaçamento de técnica e emoção durante o tratamento de crianças com diagnósticos em que a probabilidade de desfecho fatal está sempre presente. Utilizou-se abordagem qualitativa, privilegiando-se observação participante e entrevistas abertas com as médicas (neste serviço, todas as profissionais eram do sexo feminino) e mães. Buscou-se compreender a importância da comunicação que inclui expressões e controle das emoções; aspectos bioéticos que exigem sensibilidade, serenidade e verdade sobre a aproximação do final da vida; e como as médicas equilibram proximidade com as crianças e familiares e objetividade em sua atuação. Os principais resultados mostram: intensas trocas sobre ND entre as profissionais; recaída de crianças que estavam evoluindo positivamente como a notícia mais difícil; atualização da ND diante dos pacientes terminais; influência da qualidade da comunicação no tratamento; exercicío permanente de equilíbrio entre proximidade e distanciamento por parte das profissionais e evidência do insubstituível papel delas para dar segurança à família e à criança.

Abstract We present a study about the relations between pediatric oncological haematologists, mothers, and children in sharing bad news (BN) in a public hospital in Rio de Janeiro. The text emphasizes the intertwining of technique and emotions for the treatment of children with diagnoses in which the fatal outcome is always a probability. We used a qualitative approach, privileging participant observation and open interviews with oncologists (at this service all professionals were female) and mothers. We sought to understand the importance of communication which includes expressions and control of emotions; bioethical issues that require sensitivity, serenity, and truth about approaching the end of life; and how the professionals balance proximity to children and families and objectivity in their activity. The main results showed: intense exchanges on BN among professionals; relapse of children who were evolving positively as the most difficult news; constant update of BN facing terminally ill children; quality of communication influencing the treatment; professionals permanently balancing between closeness and distance from patients and evidence of the their irreplaceable role to secure the family and the child.

Hibridación in situ fluorescente: herramienta en el diagnóstico de las hemopatías malignas / Fluorescence in situ hybridization: diagnostic tool for hematological malignancies

Introducción: las neoplasias hematológicas tienen origen clonal y se caracterizan por presentar gran heterogeneidad genética. El desarrollo de la citogenética molecular a través de la hibridación in situ por fluorescencia (FISH, por su sigla en inglés) se convirtió en un avance importante en el diagnóstico citogenético de estas neoplasias. Objetivo: describir las alteraciones cromosómicas detectadas en pacientes con neoplasias hematológicas a partir de la introducción de esta técnica. Métodos: se realizó un estudio descriptivo de tipo transversal de pacientes con neoplasias hematológicas en el Laboratorio de Citogenética del Instituto de Hematología e Inmunología (IHI), en el período comprendido entre julio de 2014 y abril de 2015. Se utilizó la técnica de FISH con las sondas fluorescentes específicas. Resultados: se estudiaron 87 muestras correspondientes a diferentes tipos de neoplasias hematológicas. Con la sonda LSI BCR/ABL se observaron 18 casos positivos de leucemia mieloide crónica y los ocho pacientes con leucemia linfoide aguda fueron negativos. Se marcaron con sonda PML/RARα 17 muestras con diagnóstico de leucemia promielocítica: 10 fueron positivas. Se procesaron 8 muestras con la sonda LSI RUNX1/RUNX1T1, una resultó positiva. Dos muestras marcadas con sonda LSI RB1 (13q14) y una con LSI TP53 (17p13.1), resultaron negativas. se observó un caso positivo de deleción 7q31. Conclusiones: a pesar de que la muestra estudiada es pequeña, resulta importante reportar los primeros resultados como evidencia de la incorporación de la técnica de FISH en el IHI, lo que constituye una nueva herramienta para el diagnóstico, pronóstico y seguimiento de las neoplasias hematológicas(AU)

Introduction: hematological neoplasias have clonal origin and are characterized by great genetic heterogeneity. The development of molecular cytogenetic through fluorescence in situ hybridization (FISH) became a major advance in the cytogenetic diagnosis of these neoplasias. Aim: to describe chromosomal abnormalities detected in patients with hematological malignancies after the introduction of this technique. Methods: a descriptive cross-sectional study of patients with hematological malignancies was performed. Their bone marrow samples were processed at the Laboratory of Cytogenetics of the Institute of Hematology and Immunology, between July 2014 and April 2015. FISH technique was used along with various fluorescent probes. Results: 87 samples were studied. With LSI BCR / ABL probe, 18 samples were positive of chronic myeloid leukemia and 8 patients with diagnostic of acute lymphoblastic leukemia were negative. With PML/RARα probe 17 samples of patients with promyelocytic leukemia were labeled, 10 were positive. Eight samples were labeled with probe RUNX1 / RUNX1T1, one was positive. Two samples for LSI probes labeled RB1 (13q14) and one with LSI TP53 (17p13.1) were negative. One positive case 7q31 deletion was observed. Conclusions: despite the sample is small, we consider it important to report our first results as evidence of the incorporation of the FISH technique at the IHI, which constitutes a new tool for the diagnosis, prognosis and monitoring of hematological malignances(AU)

Acute Myeloid Leukemia With MLL Rearrangement and CD4+/CD56+ Expression can be Misdiagnosed as Blastic Plasmacytoid Dendritic Cell Neoplasm: Two Case Reports

No abstract available.

Clinical outcomes of extracorporeal membrane oxygenation support in patients with hematologic malignancies

BACKGROUND/AIMS: The clinical outcomes of patients with hematologic malignancies who were treated with extracorporeal membrane oxygenation (ECMO) after the failu re of optimal conventional therapy were determined. METHODS: The medical records of all patients administered ECMO during their stay in a medical intensive care unit of Seoul St. Mary's Hospital between February 2010 and July 2013 were reviewed retrospectively. RESULTS: In total, 15 patients with hematologic malignancies were compared to 33 immunocompetent patients with documented cardiorespiratory failure. Underlying hematologic malignancies were significantly associated with lower overall survival (0.0% vs. 24.2%, p = 0.044). Mortality was significantly associated with a higher 24 hours ECMO inspired fraction of oxygen (0.71 +/- 0.24 vs. 0.47 +/- 0.13, p = 0.015), the development of infection after ECMO (87.5% vs. 25.0%, p = 0.001), and the presence of hyperbilirubinemia (70.0% vs. 0.0%, p < 0.001). Matching of the patients based on their Acute Physiology and Chronic Health Evaluation II scores confirmed the greater risk of mortality in patients with hematologic malignancies (survival: 0.0% vs. 40.0%, p = 0.017). The mean difference in inotropic-equivalent scores after ECMO was significantly lower in the immunocompetent patients than in those with hematologic malignancies (-59.22 +/- 97.83 vs. 53.87 +/- 164.46, p = 0.026). CONCLUSIONS: Patients with hematologic malignancies who require ECMO for respiratory support have poor outcomes. The incidence of complications in these patients did not significantly differ from that in immunocompetent patients.

Neoplasia blástica de células dendríticas plasmocitoides: reporte de un caso y revisión de la literatura / Plasmacytoid dendritic cell blast neoplasia: case report and literature review

La neoplasia de células blásticas plasmocitoides dendríticas esun linfoma cutáneo poco frecuente y de mal pronóstico, que característicamenteexpresa antígenos CD4 y CD56. Clínicamentepresenta placas o nódulos de coloración violácea, únicos o múltiples.El diagnóstico se confirma con el estudio anatomopatológicoque evidencia células linfoblástica y fenotipo CD4+ y CD56+, conausencia de marcadores para células mieloides, linfoides B y T ycélulas NK. Presentamos el reporte de un caso de un paciente desexo masculino de 65 años de edad y una revisión de la literatura.

Blastic plasmacytoid dendritic cell neoplasm is a rare andhighly aggressive cutaneous lymphoma that characteristicallyexpress CD4 and CD56 antigens. Clinically presents violet colouredplaques or nodules, in a unique or multiple presentation.The diagnosis is confirmed with the anatomophatologic studythat evidence linfoblastic cells with CD4+ and CD56+ phenotype,and no mieloids, linfoids B and T and natural killers cellsmarkers. We present a case report of a 65-years-old male anda literature review.

Serum galactomannan levels in the diagnosis of invasive aspergillosis

BACKGROUND/AIMS: In this study, the sensitivity-specificity of galactomannan-enzyme immunoassay (GM-EIA) with a cut-off value of 0.5 for a single, two, or three consecutive positivity in the diagnosis of invasive pulmonary aspergillosis (IPA) in neutropenic patients with hematological malignancy was investigated. METHODS: IPA was classified as "proven," "probable," or "possible" as described in the guidelines prepared by the European Organization for Research and Treatment of Cancer and Mycoses Study Group." Serum samples were collected from the patients twice a week throughout their hospitalization. A total of 1,385 serum samples, with an average of 8.3 samples per episode, were examined. RESULTS: Based on the 165 febrile episodes in 106 patients, 80 (48.5%) were classified as IPA (4 proven, 11 probable, 65 possible) and 85 (51.5%) as non-IPA. The sensitivity/ specificity was 100%/27.1% for a single proven/probable IPA with the cut of value of GM-EIA > or = 0.5, 86.7%/71.8% for two consecutive positive results, and 73.3%/85.9% for three consecutive positive results. CONCLUSIONS: With the galactomannan levels measured twice a week, consecutive sensitivity decreased and specificity increased. Therefore, an increase may be obtained in sensitivity-specificity by more frequent monitoring of GM-EIA starting from the first day of positivity is detected.

Nutritional status of children and adolescents at diagnosis of hematological and solid malignancies

Objective: To assess the nutritional status of child and adolescent patients with cancer at diagnosis. Methods: A total of 1154 patients were included and divided into two groups: solid and hematological malignancies. The parameters used for nutritional assessment were weight, height, triceps skinfold thickness, mid-upper arm circumference, arm muscle circumference, body mass index and percentage weight loss. Results: At diagnosis, below adequate body mass index was observed by anthropometric analysis in 10.85% of the patients – 12.2% in the solid tumor group and 9.52% in the hematologic group. The average weight loss adjusted for a period of 7 days was −2.82% in the hematologic group and −2.9% in the solid tumor group. Conclusions: The prevalence of malnutrition is higher among patients with malignancies than in the general population, even though no difference was observed between the two groups...

Flow cytometric immunophenotyping of bone marrow in cats diagnosed with hematopoietic disorders

Cytological examination of bone marrow in cats, due to the large number of cells and various growth phases is somewhat complicated. The use of flow cytometric techniques and monoclonal antibodies are appropriate methods in the diagnosis of hematopoietic malignancies. The purpose of the present study is to determine cell-surface antigens for various developmental stages of feline bone marrow cells in hematopoietic disorders using flow cytometric. In this study, bone marrow cells from 4 cats with hematopoietic disorders and 2 clinically healthy cats, were labeled with 5 types of anti-feline MAbs included: CD21-like [Cr-Br], T lymphocyte subpopulation, CD-172a, Granulocyte, Pan-Leukocyte [CD45-like] and then analyzed using flow cytometric. The results revealed changes in immunophenotyping and light scatter properties compared with normal cases. The percentage of CD45, Granulocyte and CD172a markers in the bone marrow of a cat with erythroleukemia were lower compared with normal bone marrow. In a cat with myelodysplastic syndrome, scatter plot indicated an increase in the immature myeloid cells and a decrease in mature myeloid cells. It was concluded that cytological examination of bone marrow with studying dispersion studies on cells using flow cytometric and usage of a panel of antibodies such as CD21-like[Cr-Br], T lymphocyte subpopulation, CD-172a, Granulocyte, Pan-Leukocyte [CD45-like] could support the diagnosis of feline hematopoietic abnormalities

Secondary pulmonary alveolar proteinosis in hematologic malignancies

Pulmonary alveolar proteinosis [PAP], characterized by deposition of intra-alveolar PAS positive protein and lipid rich material, is a rare cause of progressive respiratory failure first described by Rosen et al. in 1958. The intra-alveolar lipoproteinaceous material was subsequently proven to have been derived from pulmonary surfactant in 1980 by Singh et al. Levinson et al. also reported in 1958 the case of 19-year-old female with panmyelosis afflicted with a diffuse pulmonary disease characterized by filling of the alveoli with amorphous material described as "intra-alveolar coagulum". This is probably the first reported case of PAP in relation to hematologic malignancy. Much progress has been made on PAP first described by Rosen which is currently classified as idiopathic or primary or autoimmune PAP. Idiopathic PAP occurs as a result of auto-antibodies directed against granulocyte-macrophage colony stimulating factor [GM-CSF] impeding the surfactant clearing function of alveolar macrophages leading to progressive respiratory failure. Whole lung lavage and GM-CSF therapy has improved outcomes in patients with idiopathic PAP. Despite major advancement in the management of hematologic malignancy and its complications, little is known about the type of PAP first described by Levinson and now known as secondary PAP; a term also used when PAP occurs due to other causes such as occupational dusts. In this article we review and analyze the limited literature available in secondary PAP due to hematologic malignancies and present a case of PAP associated with chronic lymphocytic leukemia successfully treated with bendamustine and rituximab

A Rare Case of Acute Leukemic Presentation of Blastic Plasmacytoid Dendritic Cell Neoplasm without Cutaneous Lesions

No abstract available.

Papel da imuno-histoquimica no diagnostico de adenopatias cervicais malignas / Value of immunohistochemistry in the diagnosis of malignant cervical lymph nodes

As adenopatias cervicais constituem importante condição clínica, devido à grande variedade de diagnósticos diferenciais que englobam. O uso da imuno-histoquímica tornou-se importante método auxiliar na avaliação diagnóstica de lesões linfonodais, tanto primárias como secundárias. OBJETIVO: Avaliar o uso da imuno-histoquímica no diagnóstico de malignidade nas biópsias de linfonodos. MÉTODO: Estudo retrospectivo, realizado de 2009 a 2011, com base nos resultados anatomopatológicos arquivados de biópsias de linfonodos. RESULTADOS: A casuística constituiu-se de 32 casos de biópsias de linfonodos, com uso de imuno-histoquímica em 50% (16) casos, dos quais 68,75% foram de linhagem hematogênica e 31,25%, de carcinomas. O método foi utilizado em todos os casos de linfoma. CONCLUSÃO: A imuno-histoquímica foi utilizada em 50% dos casos de biópsias de linfonodos suspeitos de malignidade, sendo em lesões de linhagem epitelial em 31,25% e, para linhagem hematopoiética, em 68,75% dos casos. .

The cervical lymph nodes are relevant due to the diversity of clinical entities. The use of immunohistochemistry is a real method to elucidate the diagnosis of adenopathy, both primary and metastatic neoplasms. OBJECTIVE: To assess the value of immunohistochemistry in the diagnosis of cervical lymph nodes malignancies. METHOD: Retrospective study of the database histopathological specimens from 2009 to 2011. RESULTS: Out of 32 biopsies of cervical lymph nodes, in 16 (50%) the immunohistochemistry was employed, being 68.75% (11) in hematological neoplasms and 31.25% (5) in carcinomas. It was used in all cases of lymphoma. CONCLUSION: The immunohistochemistry was used in 50% of the biopsies of lymph nodes under suspicion of malignancy, being 31.25% in epithelial lesions and 68.75% in lymphoproliferative lesions. .

The Utility of the Multiplex Reverse Transcriptase-Polymerase Chain Reaction Assay in the Detection of Hematologic Malignancies

No abstract available.

Epstein–Barr virus-induced haemophagocytic syndrome.

Haemophagocytic syndrome or haemophagocytic lymphohistiocytosis is a disorder of histiocytes that has sepsis-like features, combined with haemophagocytosis, cytopenias, hyperferritinaemia, hypercytokinaemia and splenomegaly. Diagnostic, therapeutic and prognostic guidelines are available for childhood (familial) haemophagocytic syndrome. The disorder is diagnosed less frequently among adults than children. We report a case of Epstein–Barr virus-induced haemophagocytic syndrome in a 23-year-old man, who responded to treatment with steroids and chemotherapy.