True-Tales Of Ten T- prolymphocytic leukemia (T-PLL) Over A Decade

Introduction:T-PLL is a mature T-cell leukemia typically presenting at stages of exponentially rising lymphocyte counts in peripheral blood, accompanied by splenomegaly and bone marrow involvement.They are rare and inherently aggressive and notoriously refractory to therapeutics.To our knowledge, this is the largest series of T-PLLs from India. Objectives; We studied Immunophenot ypic characteristics, prognostic factors, outcomes, and treatments of 10 patients with T-PLL. Methods: Out of 4500 clinically suspected chronic leukemias, during 10 years, at Kidwai Memorial Institute of Oncology, which is a state cancer institute, diagnostic flow cytometric analysis was done and leukemias were classified based on WHO 2008 criteria, along with, morphology, cytogenetics, clinical, immunophenotyping and molecular findings. Results: out of 4500 cases of Chronic lymphoprolifer ative disorders sent for flow cytometric immunophenotyping, only 10 cases were diagnosed as T-PLL, accounting for 0.4 % mature leukemias of the lymphoid lineage. multiorgan involvement was common but effusion as a presenting feature was seen in only 10% of patients. Surprisingly skin involvement was evident in more number 70% of cases. single case showed cytogenetic abnormalities, later confirmed by FISH. Conclusions: Evaluation of the immunophenotype of this entity by flow cytometry is a critical part of diagnosis and is an indispensable tool in distinguishing T -PLL from other mature T -cell lymphoid neoplasms.

Um estudo da prevalência e da caracterização da mortalidade em crianças e adolescentes por leucemia no Nordeste do Brasil / A study of the prevalence and characterization of mortality in children and adolescents due to leukemia in Northeastern Brazil / Estudio de prevalencia y caracterización de la mortalidad por leucemia en niños y adolescentes del Nordeste Brasileño

Objetivo: Caracterizar a prevalência da mortalidade por leucemias em crianças e adolescentes no Nordeste Brasileiro e sua relação com determinantes sociais da saúde no período de 2008 a 2018. Método: A pesquisa foi um estudo epidemiológico, do tipo ecológico, cujas unidades de análisede área foram os 9 (nove) estados brasileiros da Região Nordeste. Os dados desse estudo foram do tipo secundário, obtidos através do Departamento de Informática do Sistema Único de Saúde (DATASUS), provenientes do Sistema de Informação sobre Mortalidade (SIM). Para esta investigação elegeu-se, crianças e adolescentes, com faixa etária entre 0 (zero) e 19 (dezenove) anos, com óbitos no período de 2008 a 2018, cujo, a leucemia foi a causa básica mencionada na Declaração de Óbito (DO) por meios de códigos conforme a 10o Classificação Internacional de Doenças (CID-10). Resultados: foram registrados 3.025 óbitos por leucemias durante o período analisado. O CID-91 foi o primeiro colocado em número de óbitos (54,9%), em segundo lugar, CID-92 apresentando 30% das notificações e em seguida CID-95 (14,6%), CID-93 (0,3%), CID-94 (0,2%) dos óbitos. Sendo que desses, 57,6% foram do sexo masculino e 42,4% do sexo feminino. A faixa etária mais prevalente foi de 15 a 19 anos de idade (26,1%). A cor parda foi a mais autodeclarada (58,0%). O estado da Bahia foi o mais acometido por óbitos durante o período. Conclusão: O CID-91 teve maior prevalência entre óbitos notificados, que os maiores números de óbitos foram no sexo masculino com faixa etária dos adolescentes entre 15 e 19 anos e de cor parda, e que a mortalidade por leucemia ainda é um sério problema de saúde pública que pode ser reparado com medidas de prevenção e de promoção em saúde.

Objective: to characterize the prevalence of mortality of leukemia in children and adolescents in Brazilian's Northeast and the relationship with social determinants of health in the period from 2008 to 2018. Methods: It is an ecological epidemiological study that analyzed 9 (nine) Brazilian states of the Northeast Region. Data were collected through the Department of Informatics of the Unified Health System (DATASUS), from the Mortality Information System (SIM). For this, children and adolescents, aged between 0 (zero) and 19 (nineteen) years old, with deaths in the period from 2008 to 2018, that leukemia was the cause mentioned in the Death Certificate (DO) according to the 10th International Classification of Diseases (ICD-10) were included. Results: 3,025 deaths from leukemia were recorded during the analyzed period. ICD-91 was ranked first in number of deaths (54.9%), secondly, ICD-92 with 30% of notifications and then ICD-95 (14.6%), ICD-93 (0. 3%), ICD-94 (0.2%) of deaths. Regarding this, 57.6% were male and 42.4% female. The most prevalent aged group was 15 to 19 years old (26.1%). The brown color was the most self-declared (58.0%). The state of Bahia was the most affected by deaths during this period. Conclusion: The ICD-91 had a higher prevalence among notified deaths, that the highest number of deaths were in males aged between 15 and 19 years and of brown skin color, and that mortality from leukemia is still a serious problem of public health that can be repaired with prevention and health promotion measures.

Objetivo: caracterizar la prevalencia de mortalidad por leucemia en niños y adolescentes del Nordeste brasileño y la relación con los determinantes sociales de la salud en el período de 2008 a 2018. Métodos: Se trata de un estudio epidemiológico ecológico que analizó 9 (nueve) estados brasileños de la Región Nordeste. Los datos fueron recolectados a través del Departamento de Informática del Sistema Único de Salud (DATASUS), a partir del Sistema de Información de Mortalidad (SIM). Para eso, fueron incluidos niños y adolescentes, con edad entre 0 (cero) y 19 (diecinueve) años, con muerte en el período de 2008 a 2018, que la leucemia fuese la causa mencionada en el Certificado de Defunción (DME) según la 10ª Clasificación Internacional de Enfermedades (CIE-10). Resultados: Se registraron 3.025 defunciones por leucemia durante el periodo analizado. La CIE-91 ocupó el primer lugar en número de defunciones (54,9%), en segundo lugar, la CIE-92 con un 30% de notificaciones y a continuación la CIE-95 (14,6%), CIE-93 (0. 3%), CIE-94 (0,2%) de defunciones. El 57,6% eran varones y el 42,4% mujeres. El grupo de edad más prevalente fue el de 15 a 19 años (26,1%). El color castaño fue el más autodeclarado (58,0%). El estado de Bahia fue el más afectado por muertes durante este período. Conclusiones: La CIE-91 tuvo una mayor prevalencia entre las muertes notificadas, que el mayor número de muertes se produjo en varones de 15 a 19 años y de color de piel morena, y que la mortalidad por leucemia sigue siendo un grave problema de salud pública que puede ser reparado con medidas de prevención y promoción de la salud.

Técnicas de citogenética para el estudio de las leucemias / Cytogenetic techniques for the study of leukemias

Introducción: La leucemia se define como un proceso clonal de células hematopoyéticas, que se origina cuando las células sanguíneas que se producen en la médula ósea, cambian y se multiplican sin control. Esta se caracteriza por su heterogeneidad genética y se explica a través de mecanismos causados por alteraciones cromosómicas utilizados en la práctica clínica diaria como biomarcadores útiles para el diagnóstico, el pronóstico o la predicción de respuesta al tratamiento. Objetivo: Describir las técnicas de citogenética convencional y molecular para el diagnóstico y seguimiento de las leucemias. Métodos: Se realizó una revisión de la literatura en inglés y español, a través del sitio web PubMed y el motor de búsqueda Google Académico, de artículos publicados en los últimos cinco años. Se hizo un análisis y resumen de la bibliografía revisada. Análisis y síntesis de la información: En el transcurso de los años la citogenética ha proporcionado información crucial para el diagnóstico y el pronóstico de las neoplasias hematológicas. Tanto las técnicas de citogenética convencional y molecular, como la hibridación in situ fluorescente, la hibridación in situ fluorescente multicolor, el cariotipo espectral, la hibridación genómica comparada y los microarreglos, participan en el reconocimiento de alteraciones cromosómicas y de genes, así como de interacciones involucradas en el proceso de oncogénesis. Conclusiones: Las técnicas de citogenética contribuyen al diagnóstico, a la estratificación pronóstica y a la aplicación del tratamiento según el tipo o subtipo de leucemia(AU)

Introduction: Leukemia is defined as a clonal process of hematopoietic cells, which occurs when blood cells that are produced in the bone marrow change and multiply uncontrollably. This is characterized by its genetic heterogeneity and is explained through mechanisms caused by chromosomal alterations that are used in daily clinical practice as useful biomarkers for diagnosis, prognosis or prediction of response to treatment. Objective: To describe the conventional and molecular cytogenetic techniques used for the diagnosis and monitoring of leukemias. Methods: A review of the literature in English and in Spanish was carried out, in the PubMed website and using the search engine Google, for articles published in the last five years. We performed analysis and summary of the reviewed bibliography. Analysis and synthesis of information: Cytogenetics over the years has provided crucial information for the diagnosis and prognosis of hematologic malignancies. Both conventional and molecular cytogenetic techniques such as fluorescent in situ hybridization, multicolor fluorescent in situ hybridization, spectral karyotype, comparative genomic hybridization and microarrays, participate in the recognition of chromosomal and gene alterations, as well as interactions involved in the oncogenesis process. Conclusions: These cytogenetic techniques contribute to the diagnosis, prognostic stratification and application of treatment according to the type or subtype of leukemia(AU)

Perfil clínico-epidemiológico e sobrevida hospitalar dos casos de leucemia aguda em um hospital de referência em Rio Branco, Acre, 2007-2014 / Clinical epidemiological profile and survival of hospital cases of acute leukemia in a reference hospital in Rio Branco - Acre, 2007-2014

Resumo Introdução A Unidade de Assistência de Alta Complexidade em Oncologia (UNACON) permite o tratamento de leucemias agudas no Acre. Objetivo Determinar o perfil clínico-epidemiológico e a sobrevida hospitalar de leucemias agudas tratadas na UNACON/Acre entre 2007 e 2014. Método É um estudo longitudinal e retrospectivo de pacientes com leucemias agudas entre 15/06/2007 e 31/12/2014, cujos prontuários médicos forneceram dados para a análise descritiva das variáveis e posterior análise de sobrevida acumulada em 1 ano e 2 anos (método Kaplan-Meier) e comparação das curvas de sobrevida (teste de log-rank). Resultados A sobrevida para leucemias mieloides agudas (LMA) foi de 30% e 32% em 1 e 2 anos, respectivamente, com pior sobrevida para pacientes masculinos, brancos, ≥ 20 anos de idade, leucometria < 20.000 células/mm3, desidrogenase lática ≥ 600 U/dl e subtipo diferente do M3. Para leucemias linfoides agudas (LLA), a sobrevida foi de 59% e 45% em 1 e 2 anos, respectivamente, com pior sobrevida para sexo feminino, ≥ 20 anos de idade e leucometria elevada. Em pacientes abaixo de 20 anos de idade com LLA, a melhor sobrevida foi observada na faixa etária de 2 a 9 anos. Conclusão Trata-se do primeiro estudo epidemiológico de sobrevida realizado no Acre para leucemias agudas com resultados coerentes com a literatura. Contudo, novas pesquisas deverão ser realizadas.

Abstract Background The High Complexity Oncology Unit (Unidade de Assistência de Alta Complexidade em Oncologia - UNACON/Acre) allowed the treatment of acute leukemias in Acre. Objective To determine the clinical-epidemiological profile and hospital survival of acute leukemias treated at UNACON/Acre between 2007 and 2014. Method This is a longitudinal, retrospective study of patients with acute leukemias between 06/15/2007 and 12/31/2014 whose medical records provided data for descriptive analysis of the variables, and subsequent analysis of 1-year and 2-year cumulative survival (Kaplan Meier method) and comparison of survival curves (log-rank test). Results The survival for acute myeloid leukemia (AML) was 30 and 32% at 1 and 2 years, respectively, with a worse survival rate for males, white, age ≥20 years, leukometry <20,000 cells/mm3, lactic dehydrogenase ≥600 U/dl and subtype different from M3. For acute lymphoid leukemias (ALL), survival was 59 and 45% at 1 and 2 years, respectively. Female gender, age ≥20 years, and high leukometry had worse survival. For patients <20 years with ALL, better survival was observed in the age group of 2-9 years. Conclusion This is the first epidemiological study of survival in Acre for acute leukemias with results consistent with the literature. However, new studies should be performed.

Clinical features and prognosis of children with acute leukemias of ambiguous lineage under different diagnostic criteria / 中国当代儿科杂志

OBJECTIVES@#To study the clinical features and prognosis of children with acute leukemias of ambiguous lineage (ALAL) under different diagnostic criteria.@*METHODS@#A retrospective analysis was performed on the medical data of 39 children with ALAL who were diagnosed and treated from December 2015 to December 2019. Among the 39 children, 34 received treatment. According to the diagnostic criteria for ALAL by World Health Organization and European Group for the Immunological Characterization of Leukemias, the 39 children were divided into two groups: ALAL group (@*RESULTS@#The 34 children receiving treatment had a 3-year event-free survival (EFS) rate of 75%±9% and an overall survival rate of 88%±6%. The children treated with acute myeloid leukemia (AML) protocol had a 3-year EFS rate of 33%±27%, those treated with acute lymphoblastic leukemia (ALL) protocol had a 3-year EFS rate of 78%±10%, and those who had no remission after induction with AML protocol and then received ALL protocol had a 3-year EFS rate of 100%±0% (@*CONCLUSIONS@#ALL protocol has a better clinical effect than AML protocol in children with ALAL, and positive MRD after induction therapy suggests poor prognosis. Hyperleukocytosis and adverse genetic changes are not observed in children with myeloid expression, and such children tend to have a good prognosis, suggesting that we should be cautious to take it as ALAL in diagnosis and treatment.

Frecuencia de aberraciones moleculares en pacientes cubanos con leucemia mieloide aguda / Frequency of molecular disorders in Cuban patients with acute myeloid leukemia

Introducción: En el Instituto de Hematología e Inmunología se realiza el estudio molecular de las leucemias mieloides agudas (LMA). Para las leucemias mieloides agudas no promielocíticas (LPM) se determinan cuatro biomarcadores: los genes de fusión RUNX1-RUNX1T1 y CBF(-MYH11, la duplicación interna en tándem del gen FLT3 (DIT FLT3) y la mutación A del gen NPM1 (NPM1-A). Objetivo: Determinar la frecuencia de estos cuatro biomarcadores, en pacientes cubanos con leucemias mieloides agudas primaria no promielocíticas. Métodos: Se incluyeron 91 pacientes entre niños y adultos, estudiados en el Instituto durante tres años desde el debut. A partir de ARN de sangre medular se obtuvo ADN complementario por transcripción inversa; se amplificaron los fragmentos correspondientes mediante la reacción en cadena de la polimerasa y el producto se analizó por electroforesis capilar. Resultados: El RUNX1-RUNX1T1 apareció en el 24,2 por ciento, fue más frecuente en los pacientes pediátricos y disminuyó significativamente con la edad. El CBFβ-MYH11 solo se encontró en adultos (4,8 por ciento). La NPM1-A con 41 por ciento fue mayoritaria entre los adultos. La DIT FLT3 se observó en el 21,6 por ciento y no mostró relación con la edad. NPM1-A y DIT FLT3 fueron las aberraciones con mayor presencia simultánea. Conclusiones: Por primera vez se describe la frecuencia de los cuatro biomarcadores moleculares en los pacientes cubanos con leucemias mieloides agudas primaria no promielocíticas; su comportamiento fue similar a lo descrito por otros autores, aunque se encontraron algunas particularidades(AU)

Introduction: At the Institute of Hematology and Immunology, the molecular study of acute myeloid leukemias (AML) is carried out. For nonpromyelocytic acute myeloid leukemias, four biomarkers are determined: the RUNX1-RUNX1T1 and CBF(-MYH11 fusion genes, the internal tandem duplication of the FLT3 gene (DIT FLT3), and the A mutation of the NPM1 gene (NPM1-A). Objective: To determine the frequency of these four biomarkers in Cuban patients with nonpromyelocytic primary acute myeloid leukemias. Methods: 91 patients were included, children and adults, who were studied at the Institute for three years from their disease debut. Complementary DNA was obtained from medullary blood RNA by reverse transcription. The corresponding fragments were amplified by polymerase chain reaction and the product was analyzed by capillary electrophoresis. Results: RUNX1-RUNX1T1 appeared in 24.2 percent; it was more frequent in pediatric patients and decreased significantly with age. CBFβ-MYH11 was found only in adults (4.8 percent). NPM1-A, accounting for 41 percent, represented the majority among adults. FLT3 DIT was observed in 21.6 por ciento and was not related to age. NPM1-A and DIT FLT3 were the disorders with the greatest concurrence. Conclusions: For the first time, the frequency of the four molecular biomarkers is described in Cuban patients with primary non-promyelocytic acute myeloid leukemias. Its characterization was similar to that described by other authors, although some peculiarities were found(AU)

Caracterización de las leucemias en niños en Cuba (2006-2015) / Characterization of leukemias in Cuban children (2006-2015)

Introducción: La leucemia es el cáncer más frecuente en edades pediátricas. La leucemia linfoide aguda (LLA) representa el 23 por ciento de los diagnósticos de cáncer en niños menores de 15 años y el 75 por ciento de todas las leucemias. Más del 90 por ciento de las leucemias mieloides agudas (LMA) en la niñez son agudas y el resto son crónicas. Objetivo: Caracterizar las leucemias agudas de la edad pediátrica en Cuba. Métodos: Estudio descriptivo, longitudinal y retrospectivo. La muestra fue de 799 niños de toda Cuba, los cuales fueron atendidos en los siete hospitales pediátricos acreditados en el país para el tratamiento de leucemias agudas, entre enero 2006 y diciembre de 2015. Como medida de resumen para las variables cuantitativas se utilizaron la media y desviación estándar. Para todas las variables cualitativas se calcularon los porcentajes de cada grupo. Resultados: Las tasas de incidencia fueron de 2,9 x 100 000 niños. El 95 por ciento fueron leucemias agudas (70,6 por ciento linfoides), con el 34,9 por ciento de leucemia promielocíticas (LPM) en las LMA. Predominó el sexo masculino y la edad promedio al diagnóstico fue de 7,1 años. El porcentaje de remisión completa en las LLA fue de 91 por ciento y en las LMA, de 66,7 por ciento. La frecuencia de recaídas de la enfermedad fue de 25,1 por ciento en las LLA, 13,7 por ciento en las LPM y 45 por ciento en las otras mieloides. La sobrevida global y libre de eventos fue de 89 por ciento y 63 por ciento en los niños con LLA, 64 por ciento y 62 por ciento en la LPM, 38 por ciento y 36 por ciento en la LMA no promielocítica, respectivamente. Conclusiones: La frecuencia y distribución etaria de las leucemias en niños cubanos son similares a lo descrito a nivel. Los resultados del tratamiento en las leucemias agudas de manera general pueden considerarse como buenos(AU)

Introduction: Leukemia is the most frequent cancer in pediatric ages. Acute lymphoid leukemia (ALL) accounts for 23 percent of cancer diagnoses in children at ages under 15 years and 75 percent of all leukemias. More than 90 percent of childhood acute myeloid leukemias (AML) are acute and the rest are chronic. Objective: To characterize acute leukemias in pediatric age in Cuba. Methods: Descriptive, longitudinal and retrospective study. The sample was 799 children from all over Cuba, who were treated in any of the country's seven pediatric hospitals accredited for the treatment of acute leukemias, between January 2006 and December 2015. As a summary measure for the quantitative variables, the mean and standard deviation were used. For all qualitative variables, the percentages of each group were calculated. Results: Incidence rates were 2.9 per 100,000 children. 95 percent were acute leukemias (70.6 percent lymphoid), with 34.9 percent corresponding to promyelocytic leukemia (PML) in AML. The male sex predominated and the average diagnosis age was 7.1 years. The percentage of complete remission in ALL was 91 percent, and 66.7 percent in AML. The frequency of disease relapse was 25.1 percent in ALL, 13.7 percent in LPM, and 45 percent in other myeloid leukemias. Overall and event-free survival was 89 percent and 63 percent in children with ALL, 64 percent and 62 percent in LPM, 38 percent and 36 percent in non-promyelocytic AML, respectively. Conclusions: Frequency and age distribution of leukemias in Cuban children are similar to that described worldwide. The results of treatment in acute leukemias in general can be considered as good(AU)

Prognostic significance of receptor for hyaluronan acid-mediated motility (CD168) in acute pediatric leukemias - assessment of clinical outcome, post induction, end of treatment and minimal residual disease

ABSTRACT Introduction: The extracellular matrix protein hyaluronan acid plays an active in role in tumor cell proliferation and invasion. Hyaluronan acid receptors, namely CD168 or the receptor for hyaluronan acid-mediated motility (RHAMM) and CD44 have been implicated in promoting malignancy. There is a lacuna in data on the expression of the receptor in pediatric leukemias. Methods: Pediatric patients with acute leukemia who were diagnosed, treated and followed up in our center were enrolled. The bone marrow biopsies performed prior to treatment were subjected to immunohistochemical staining (54 biopsies: acute lymphoblastic leukemia - 45, acute myeloid leukemia - 9). Blast counts were carried out at diagnosis, end of the induction phase and end of chemotherapy, the minimal residual disease was assessed and follow up details were collected. Positivity was correlated with initial blast count, post-induction blast count, minimal residual disease and patient survival. Results: There was no correlation between the initial blast count and the percentage of blasts with RHAMM expression. The positive correlation between percentage of blasts expressing RHAMM and the post-induction blast count was moderate in acute myeloid leukemia (0.74) and mild in acute lymphoblastic leukemia (0.48). There was a statistically significant difference in RHAMM expression between the two minimal residual disease risk groups (p-value = 0.012) with a negative prognostic effect of RHAMM expression. Moreover, a negative prognostic effect of RHAMM expression was noted when patient survival was considered. Conclusion: This study shows that blasts in acute myeloid leukemia show more RHAMM positivity than those of acute lymphoblastic leukemia indicating the aggressive nature of this type of leukemia. In acute leukemias, patients with high percentages of RHAMM-positive blasts had more post-induction blasts, blasts in minimal residual disease and poorer prognosis.

Molecular epidemiology of acute leukemia in children: causal model, interaction of three factors-susceptibility, environmental exposure and vulnerability period / Epidemiología molecular de la leucemia aguda en niños. Modelo causal en el que intervienen tres factores: susceptibilidad, exposición ambiental y periodo de vulnerabilidad

Acute leukemias have a huge morphological, cytogenetic and molecular heterogeneity and genetic polymorphisms associated with susceptibility. Every leukemia presents causal factors associated with the development of the disease. Particularly, when three factors are present, they result in the development of acute leukemia. These phenomena are susceptibility, environmental exposure and a period that, for this model, has been called the period of vulnerability. This framework shows how the concepts of molecular epidemiology have established a reference from which it is more feasible to identify the environmental factors associated with the development of leukemia in children. Subsequently, the arguments show that only susceptible children are likely to develop leukemia once exposed to an environmental factor. For additional exposure, if the child is not susceptible to leukemia, the disease does not develop. In addition, this exposure should occur during a time window when hematopoietic cells and their environment are more vulnerable to such interaction, causing the development of leukemia. This model seeks to predict the time when the leukemia develops and attempts to give a context in which the causality of childhood leukemia should be studied. This information can influence and reduce the risk of a child developing leukemia.

La leucemia aguda representa una enfermedad con una enorme heterogeneidad morfológica, citogenética, molecular y polimorfismos genéticos asociados con la susceptibilidad. Presenta factores causales asociados con el desarrollo de la misma. Particularmente, cuando tres fenómenos se conjuntan, traen como resultado el desarrollo de la leucemia aguda. Estos fenómenos son la susceptibilidad, la exposición ambiental y un periodo que, para este modelo, ha sido denominado el periodo de vulnerabilidad. El presente marco teórico muestra cómo los conceptos de la epidemiología molecular han permitido establecer una referencia a partir de la cual es más factible identificar los factores ambientales relacionados con el desarrollo de la leucemia en niños. Posteriormente se muestran los argumentos para predecir que solo los niños susceptibles probablemente desarrollarán leucemia una vez que se exponen a un factor ambiental. Por lo que, por más exposición, si el niño no es susceptible a la leucemia, no la desarrollará. Además, esta exposición debe ocurrir durante una ventana de tiempo durante el cual las células hematopoyéticas y su entorno son más vulnerables, para que dicha interacción provoque el desarrollo de leucemia. Este modelo pretende predecir el momento en el cual se desarrollará la leucemia y trata de dar un contexto en el que la causalidad de la leucemia en niños deberá ser estudiada. A través de esto se podrá influir y disminuir el riesgo de que un niño desarrolle leucemia.

Prevalence and spectrum of anemia among 3920 pediatric in-patients A cross sectional study.

Background: Anemia is one the common hematological laboratory abnormality in pediatric practice. To this reason we have studied the prevalence and spectrum of anemia among pediatric in-patients whom were admitted in a tertiary care hospital in Andhra Pradesh. Materials and methods: The present cross sectional study was done in the Department of Pathology at tertiary care hospital for two years. All pediatric patients with anemia due to clinically suspected or proven cases of leukemia were included. Patients with other causes of anemia such as chronic infection and systemic disorders were excluded. The study was based on peripheral smear examination and bone marrow aspiration cytology reports. Clinical details of these patients were recorded. Anemia and leukemias were grouped and classified based on the morphological type of the FAB classification. Data was extracted from patient case records and laboratory reports. Data management was done using Microsoft excel spreadsheet and statistical analysis using SPSS-16. Actual numbers and percentages were used as descriptive statistics. Results: A total of 3920 patients were admitted in pediatric ward. Among them, 212/3920 (5.4%) were being the hematological diseases, 170/212 (80.2%) being anemic due to causes other than leukemia’s and 42/212 (19.8%) were anemia due to leukemia’s. Among the 170 cases, the anemia due to Iron, Folate and B12 deficiency was 163 cases (76.88%) of total cases and seven cases (3.31%) were hemolytic anemia’s. Among these 42 leukemia cases, acute lymphoblastic leukemia’s were 32 (76.2%) AML were 19.04% (8) and CML was 4.76% (2). Conclusion: The commonest type of anemia noticed was the microcytic hypochromic anemia due to nutritional deficiency, followed by macrocytic anemia. 20% anemias were due to leukemias. The Neeraja P, Reddy CB. Prevalence and spectrum of anemia among 3920 pediatric in-patients – A cross sectional study. IAIM, 2016; 3(4): 11-14. Page 12 commonest acute leukemia noted was ALL (L1 type). There is seasonal raise of anemia incidences was also noticed between June and September, of which cause is not known. Nutritional anemias can be preventable and treatable effectively through better mother and child health care policies.

Metodología y aplicaciones de la citometría de flujo para el inmunofenotipaje de las leucemias agudas / Methodology and applications of flow cytometry for immunophenotyping of acute leukemias

La citometría de flujo (CMF) es una técnica de avanzada, altamente sensible y automatizada, que se emplea para el inmunofenotipaje de las células normales y leucémicas. En este artículo se muestran los principales aspectos metodológicos a tener en cuenta para un mejor desarrollo e interpretación del inmunofenotipo por CMF, entre los que se encuentran: tipo, cantidad, conservación y transportación de la muestra, uso de anticoagulantes, empleo de anticuerpos monoclonales y fluorocromos, lisado de hematíes, fijación celular, así como la calibración y compensación de la auto-fluorescencia. Finalmente, se exponen las principales aplicaciones de esta metodología para definir el estado de maduración celular leucémico, clasificar las leucemias agudas en distintos subtipos inmunológicos, identificar subgrupos de mal pronóstico y detectar fenotipos aberrantes. Todo lo anterior resulta de gran utilidad para el diagnóstico de la enfermedad mínima residual que permite estratificar a los pacientes en diferentes grupos de riesgo e individualizar el tratamiento antileucémico(AU)

Flow cytometry (FCM) is an advanced, highly sensitive and automated technique used for immunophenotyping of normal and leukemic cells. The main methodological aspects to consider for better development and interpretation of immunophenotyping by FCM are shown in this article. Among them are: type, quantity, storage and transportation of the sample, use of anticoagulants, use of monoclonal antibodies and fluorochromes, erythrocyte lysate, cell attachment, calibration and auto-fluorescence compensation. Finally, the main applications of this methodology are given for defining the state of leukemic cell maturation, acute leukemias rank in different immunological subtypes, poor prognosis subgroups and to identify and detect aberrant phenotypes, which are useful for the diagnosis of minimal residual disease, allowing to stratify patients into different risk groups and thus to identify the anti-leukemic treatment(AU)

Leucemias na clínica pediátrica / Leukemias in Pediatric Practice

Os autores discutem a importância e frequência das leucemias na infancia, discorrendo sobre o quadro clinico, diagnóstico e tratamento das leucemias agudas, moléstia crônica e bifenotípica.

Leucemias agudas en ancianos de la provincia de Santiago de Cuba / Acute leukemias in the elderly from Santiago de Cuba province

Se realizó un estudio descriptivo y retrospectivo de 64 pacientes mayores de 60 años con leucemia aguda, atendidos en el Servicio de Hematología del Hospital General Docente "Dr. Juan Bruno Zayas Alfonso" de Santiago de Cuba, durante el quinquenio 2006-2011, para determinar las principales características clínicas y hematológicas en el momento del diagnóstico, así como la supervivencia global de los afectados, aunque los tratamientos administrados no tenían criterio curativo. La edad promedio de los ancianos fue de 70 años, en un rango etario de 60 a 90; en tanto, la variedad no linfoblástica representó 98,4 %, y todos los pacientes presentaron anemia y trombocitopenia como alteraciones hematológicas, con incremento en los requerimientos transfusionales. De igual forma, la presencia de blastos en la sangre periférica se demostró en 50 % y la hiperleucocitosis en 59,4 %, mientras las principales causas de muerte estuvieron relacionadas con la hemorragia cerebral y la progresión de la enfermedad con la infiltración multiorgánica, lo cual condujo a una supervivencia muy corta de los integrantes de la serie...

A descriptive and retrospective study of 64 patients older than 60 years with acute leukemia, assisted in the Hematology Service of "Dr. Juan Bruno Zayas Alfonso" Teaching General Hospital in Santiago de Cuba was carried out during the five year period 2006-2011, to determine the main clinical and hematological characteristics at the moment of diagnosis, as well as the global survival of those affected, although the administered treatments had no healing criterium. The average age of the elderly was 70 years, in an age range of 60 to 90; while the non lymphoblastic variety represented 98.4%, and all the patients presented anemia and thrombocytopenia hematological changes, with increment in the transfusional requirements. Likewise, the blastos presence in the peripheral blood was demonstrated in 50% and the hyperleucocytosis in 59.4%, while the main causes of death were related to the cerebral hemorrhage and the progression of the disease with the multiorganic infiltration, which led to a very short survival of the members of this series...

Complicaciones infecciosas en el período inmediato precoz postrasplante de progenitores hematopoyéticos / Infectious complications in the immediate early post-transplantation of hematopoietic stem cells

Se realizó un estudio retrospectivo-descriptivo en el Servicio de Hematología del Hospital Clinicoquirúrgico Hermanos Ameijeiras desde enero de 1985 hasta diciembre de 2008, para determinar las complicaciones infecciosas en pacientes sometidos a trasplante de progenitores hematopoyéticos en el período precoz postrasplante (0-30 d). De los 179 trasplantes realizados, 127 fueron autólogos, 45 alogénicos y 7 mini-trasplantes. Las principales indicaciones fueron linfomas no Hodgkin, en 54 pacientes (30,2 por ciento), leucemias agudas no linfoblásticas, en 53 (29,6 por ciento) y linfomas de Hodgkin, en 26 (14, 5 por ciento). El esquema de acondicionamiento usado mayoritariamente fue ciclofosfamida, con etopósido o sin Úl, + irradiación corporal total. Se detectaron complicaciones infecciosas en 176 de los trasplantes realizados (98,3 por ciento), su aparición fue entre los días 4 y 7 después del trasplante y se relacionó con un conteo promedio de leucocitos menor de 500 x 10(9) /L y un conteo absoluto de neutrófilos menor de 100 x mm³. La forma clínica de presentación más frecuente fue la bacteriemia seguida de infecciones de piel y mucosas. Los gérmenes aislados con mayor frecuencia fueron el estafilococo coagulasa negativo, el género Candida y la Pseudomona aeruginosa. La antibioticoterapia empírica utilizada fue cefalosporina de tercera generación + aminoglucósido + vancomicina. La mortalidad general fue 8,9 por ciento (16/179), la sepsis fue la causa directa del fallecimiento en 7 casos (43,7 por ciento) con mayor incidencia en los trasplantes alogénicos, y las bacterias gramnegativas, las mayormente implicadas

A retrospective and descriptive study was carried out in the hematological service of Hermanos Ameijeiras clinical and surgical hospital from January 1985 to December 2008, in order to determine the infectious complications found in patients with hematopoietic stem cell transplants in the early post-transplantation period (0 to 30 days). Out of 179 transplants, 127 were autologous, 45 allogenic and 7 mini-transplants. The main indications for transplantation were non-Hodgking's lymphomas in 54 patients (30.2 percent), acute non-lymphoblastic leukemia in 53 (29.6 percent) and Hodgkin's lymphomas in 26 patients (14.5 percent). The mostly used conditioning schedule was cyclophosphamide with/without etoposide plus total body irradiation. One hundred seventy six transplants (98.3 percent) presented with infectious diseases; they occurred on the 4th through the 7th days after the transplantation and were related to average leukocyte count below 500 x 10(9)/L and absolute neutrophil count lower than 100 x mm³. The most frequent clinical presentation was bacteremia followed by skin and mucosal infections. The most commonly isolated germs were negative coagulase staphylococci, Candida genus and Pseudomona aeruginosa. The empirical antibiotic therapy was third generation cephalosporine plus aminoglucoside plus vancomycin. The general mortality rate was 8.9 percent (16 out of 179) and sepsis directly caused the death of 7 cases (43.7 percent), being the higher incidence in allogenic transplants, and the Gram negative bacteria were mostly involved

Aplicación de un modelo pronóstico para predecir la evolución de la neutropenia febril en niños con leucemias agudas / A new model applied in prediction of febrile neutropenia in children with acute leukemias

Se realizó estudio analítico retrospectivo de casos controles en 62 episodios de neutropenia febril presentados por pacientes con hemopatías malignas admitidos en el Instituto de Hematología e Inmunología durante los años 2005 y 2006. Los episodios de neutropenia febril se dividieron en 2 grupos a partir de su evolución favorable (56 %) o desfavorable (44 %) y se compilaron los parámetros clínicos y de laboratorio en ambos grupos. Los factores que mayor asociación mostraron con la evolución desfavorable fueron la presencia de comorbilidad (sepsis, deshidratación, hipoxia, hipovolemia, mucositis severa), el diagnóstico de leucemia aguda no linfoblástica, el uso de quimioterapia en los 7 días previos al inicio de la fiebre, el compromiso del estado general, y el conteo absoluto de neutrófilos menor de 100 x mm³. Los pacientes con neutropenia febril de evolución desfavorable presentaron entre 3 y 5 factores de riesgo, mientras que los de evolución satisfactoria tuvieron menos de 2 factores de mal pronóstico. El valor predictivo positivo del modelo pronóstico fue del 91,4 % y el negativo del 92,6 %, con una sensibilidad y especificidad del 94,1 % y 89,3 %, respectivamente.

A retrospective and analytical control cases study was carried out in 62 episodes of febrile neutropenia present in patients with malignant blood disease admitted in Hematology and Immunology Institute during 2005 and 2006. Above episodes were divided in 2 groups from its favorable course (56%) or unfavorable (44%) as well as the laboratory and clinical parameters compiled in both groups. Factors with higher association related to unfavorable course were the comorbidities presence (sepsis, dehydration, hypoxia, hypovolemia and severe mucositis), diagnosis of non-lymphoblastic, use of chemotherapy during the 7 previous days to fever onset, general status involvement, and the neutrophil absolute count lower than 100 x mm³. Patients presenting with a unfavorable course febrile neutropenia had two poor diagnosis factors. The positive predictive value of prognostic model was of 91,4% and the negative one was of 92,6% with a sensitivity and specificity of 94,1% and 89,3%, respectively.

Gastrointestinal manifestations as initial presentation of acute leukemias in children and adolescents / Manifestaciones gastrointestinales como presentación de las leucemias agudas en niños y adolescentes

OBJECTIVE: this study aimed to determine the prevalence and characteristics of gastrointestinal manifestations on initial clinical presentation of acute leukemias (AL) in childhood. MATERIAL AND METHODS: this is a retrospective and descriptive study that assessed medical records of 354 patients with AL from January 1995 to December 2004. RESULTS: acute lymphoid leukemia (ALL) was diagnosed in 273 (77.1%) patients and acute non-lymphocytic leukemia (AML) in 81 (22.9%). There were 210 males (59.4%) and 144 females (40.6%). The most common presenting features were: abdominal pain (19.5% in ALL and 11.8% in AML), nausea and vomiting (14.9 in ALL and 14% in AML), abdominal distention (18.5 in ALL and 8.6% in AML; p 0.024), constipation (5% in ALL and 6.5% in AML), diarrhea (3.6% in ALL and 11.8% in AML; p 0.03%), and gastrointestinal bleeding (7.9% in ALL and 9.7% in AML). Ultrasound scanning was made in 61.1% and hepatomegaly was found on 33.6% and esplenomegaly on 28.5% of the patients with AL. Seventy-seven (21.7%) and 15 (4.2%) patients received nonsteroidal anti-inflammatory drugs and glucocorticoids before the diagnostic of AL. An association is well-defined between abdominal symptoms like nausea, vomiting and pain and use of this therapy but this association did not occurred clearly in this study. CONCLUSIONS: gastrointestinal symptoms are not very well-documented as initial manifestation of leukemia in children and should be considered on the differential diagnosis of gastrointestinal symptoms of unknown etiology in children.

Objetivo: el objetivo del estudio fue determinar la prevalencia y las características de las manifestaciones gastrointestinales en la presentación clínica inicial de las leucemias linfoides agudas (LLA) en la infancia. Materialy métodos: se trata de un estudio descriptivo y retrospectivo que evaluó los registros médicos de 354 pacientescon LLA de enero de 1995 a diciembre de 2004. Resultados: la (LLA) ha sido diagnosticada en 273 (77,1%) pacientes y leucemia mieloide aguda (LMA) en 81 (22,9%). Hubo 210 niños (59,4%) y 144 niñas (40,6%). Los síntomas más comunes de presentaciónhan sido los siguientes: dolor abdominal(19,5% en LLA y 11,8% en el LMA), náuseas y vómitos (14,9 en LLA y 14% en LMA, P 0.024), distensión abdominal (18,5 en LLA y 8,6% en LMA, p 0,024), estreñimiento (5% en LLA y 6,5% en LMA), diarrea (3,6% en LLA y 11,8% en LMA, p 0,03%) y hemorragia gastrointestinal (7,9% en LLA y 9,7% enLMA). La ecografía fue realizada en 61,1% de los pacientes encontrándose hepatomegalia en 33,6% y esplenomegalia en 28,5% con LLA. Setenta y siete (21,7%) y 15 (4,2%) pacientes recibieron los fármacos antiinflamatorios no esteroides y glucocorticoides antes del diagnóstico de LLA. Hay una asociación bien definidaentre síntomas abdominales como náuseas, vómitos y dolor y el uso de esta terapia pero esta asociación no seprodujo claramente en este estudio. Conclusiones: las manifestaciones gastrointestinales no están bien documentadas como manifestaciones iniciales de la leucemia en los niños y debe considerarse en el diagnóstico diferencial de los síntomas gastrointestinales de etiología desconocida en estas edades.

Mortalidad por leucemias en menores de 20 años: México 1998-2002 / Leukemia mortality on people under 20 years old: Mexico 1988-2002

Introducción. Las leucemias son el cáncer más frecuente durante la infancia. El estudio pretende describir la mortalidad por leucemias en menores de 20 años en México. Material y métodos. A partir del Sistema Estadístico y Epidemiológico de las Defunciones se calcularon tasas específicas por edad, género y entidad federativa. Se estimó la tasa media de mortalidad anual (TMMA) por estado, y la tasa truncada estandarizada por edad de mortalidad. La estandarización fue por el método directo y el error estándar por la aproximación de Poisson, los intervalos de confianza (IC) fueron de 95%. En la elaboración de la razón estandarizada de mortalidad (REM) se utilizó la tasa nacional como referencia. Se calculó la proporción de cambio anual estatal y nacional con IC al 95%, además se estimaron las tendencias nacionales y estatales de 1998 a 2002 por medio de la regresión de Poisson. Resultados. La mortalidad por leucemias representó 51.1%. La razón hombre/mujer fue de 1.3. Los grupos de edad más afectados fueron los de 5-9 y 10-14 años, ambas con TMMA de 27.7 por 10(6) habitantes. La REM para Quintana Roo y Puebla fueron significativas. En cuanto a la tendencia Tlaxcala presentó un incremento y Baja California Sur un decremento, ambos fueron estadísticamente significativos. Conclusiones. La mortalidad por leucemias en menores de 20 años representa un problema de salud pública nacional, por lo que el diagnóstico temprano y tratamiento específico deben ser de alta prioridad.

Introduction. Leukemias are the most frequent form of cancer in childhood and adolescence. This study describes the mortality rate for individuals under 20 years of age with a primary diagnosis of leukemia in Mexico over a 15 year period, from 1988-2002. Material and methods. Specific mortality rates were calculated according to age, gender and state of origin based on data provided by a National Epidemiological Mortality Reporting System (SEED). The median annual mortality rate and age adjusted mortality rate were estimated for each state in Mexico. The direct method was used for standardization and standard error with 95% confidence intervals were also calculated. The national mortality rate was used as a reference to estimate the standardized mortality rate. State annual change and trends were calculated from 1988 to 2002 by Poisson regression. Results. The leukemia mortality rate during the study period was 51.1%; the male/female ratio was 1.3 and the predominant age group ranged from 10 to 14 years of age. The median annual mortality rate of 27.7 per 10(6) inhabitants. Conclusions. Leukemia mortality in children and adolescents under 20 years of age represents a major public health problem in Mexico, early diagnosis and specific treatment must be considered high priority.

Mordern treatment of myelodysplastic syndrome / 中国实用内科杂志

Myelodysplastic syndrome (MDS) is a clonal disorders characterized by ineffective hematopoiesis,which can lead to either fatal cytopenias or acute myelogenous leukemias (AML).MDS mainly occurs in the elderly population.The current use of allogeneic hematopoietic stem cell transplantation for MDS patients is the only medical care,but not available for patients who are above 60 years.Recently,several new biological and chemotherapeutic agents have been used in the treatment of MDS alone or jointlly,which may result in the improvement of hematologic parameters and the prevention of disease progression.

Clinicohematologic Observations of Acute Leukemias with Hyperleukocytosis / 대한내과학회지

OBJECTIVES: It is well known that Acute Leukemic patients with Hyperleukocytosis (ALH, leukocyte count>or=100,000/micro L) have poor prognosis. This is indebted in fatal complications arising from cerebral and pulmonary leukostasis. To investigate the factors influence on the prognosis of these patients, we have analyzed age, sex, laboratory findings and complications and their relationship to remission rate. METHODS: Retrospective evaluation was done from January 1985 to March 1994 on fifty-four patients with ALH. We excluded secondary leukemias transformed from chronic myelogeneous leukemia, relapsed acute leukemia and myelodysplastic syndrome in this study. The prognostic factors associated with early death were also evaluated. RESULTS: 1) Hyperuricemia and incidence of central nervous system and respiratory symptoms were higher in acute myelogeneous leukemia (AML) with hyperleu-kocytosis than in acute lymphocytic leukemia (ALL), 2) Twenty-two of fifty-four patients had complete remission by remission induction chemotherapy. Remission rate was 41%, median duration of remission was 26 weeks and 1 year survival rate was 11%. 3) There were no differences in remission rate between male and female and higher WBC group (WBC>or=200,000/micro L) and lower WBC group (WBC 100,000~200,000/micro L). 4) The group with better performance status (ECOG score1-2), younger (age below 40) and higher hemoglobin level (Hb>or=10g/dL) had higher remission rate. The group of AML and with hepatomegaly had lower remission rate than the group of ALL and without hepatomegly. 5) Early death rate of AML was higher than that of All. Infection was the most common cause of early death in both AML and ALL. 6) Early death rate between the two groups managed with and without leukapheresis was not different. CONCLUSIONS: This result reveals that acute leukemia with hyperleukocytosis is grave disease, especially the patients with poor performance status (ECOG score: 3-4), older age above 40 and severe anemia (Hb<10g/dL) have poor prognosis, The group of AML and with hepatomegaly showed worse prognosis than the group of ALL and without hepatomegaly.