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1.
Rev. cuba. hematol. inmunol. hemoter ; 37(3): e1445, 2021. tab, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1341399

ABSTRACT

Introducción: Durante el tratamiento de inducción de la leucemia linfoide aguda en niños no siempre se identifican las reacciones adversas a medicamentos. Objetivo: Describir los eventos adversos y las reacciones adversas a medicamentos durante el tratamiento de inducción de la leucemia linfoide aguda, en niños tratados en el Instituto de Hematología e Inmunología de Cuba, durante 2012-2017. Método: Estudio observacional, descriptivo, transversal, de series de casos en farmacovigilancia, se utilizó la farmacovigilancia activa. Variables: sexo, edad, grupo pronóstico, semana de tratamiento, tipo de evento adverso, sistema de órgano afectado, severidad e imputabilidad. La información se obtuvo del registro nacional del protocolo ALLIC-BFM 2009 y las historias clínicas. Resultados: Se incluyeron 69 niños, 55,1 por ciento (38 casos) fueron masculinos, 56,5 por ciento (39 niños) tenía entre uno y seis años. El 52,2 por ciento (36 pacientes) pertenecían al grupo pronóstico intermedio. Se registraron 471 eventos adversos. El 50,5 por ciento (238/471) ocurrió en la primera semana de tratamiento. Los más frecuentes: anemia (17,8 por ciento; 84/471), neutropenia (16,1 por ciento; 76/471) y trombocitopenia (15,9 por ciento; 75/471). Los sistemas de órganos más afectados: hemolinfopoyético (57,54 por ciento; 271/471) y gastrointestinal (15,71 por ciento; 74/471). El 93,2 por ciento (439/471) se clasificó en reacciones adversas posibles. Según gravedad el 72,4 por ciento (330/456) fueron moderadas y el 27,4 por ciento (125/456) graves. Conclusiones: Todos los casos presentaron eventos adversos, predominaron las alteraciones hematológicas y los eventos reportados para fármacos incluidos en la quimioterapia. Se identificaron reacciones adversas clasificadas como posibles, con predominio de las moderadas y graves(AU)


Introduction: During the induction treatment of acute lymphoid leukemia in children, adverse drug reactions are not always identified. Aims: Describe the demographic and clinical characteristics of children with acute lymphoid leukemia who receive induction treatment at the Institute of Hematology and Immunology between 2012-2017. Characterize adverse events that occur during induction treatment. Describe adverse drug reactions during induction. Methods: Observational, descriptive, cross-sectional study of case series in pharmacovigilance, used active pharmacovigilance. Variables: sex, age, prognosis group, week of treatment, type of adverse event, organ system affected, severity and imputability. The information was obtained from the national register of the ALLIC-BFM 2009 protocol and the medical records. Results: 69 children were included, 55.1 percent belonged to the male sex, 56.5 percent were between one and six years old. 52.2 percent (36 children) belonged to the intermediate prognosis group. 471 events were recorded. 50.5 percent occurred in the first week of treatment. The most frequent: anemia (17.8 percent), neutropenia (16.1 percent) and thrombocytopenia (15.9 percent). The most affected organ systems: hemolinfopoietic (57.5 percent) and gastrointestinal (15.7 percent). According to the severity, 72.4 percent were moderate and 27.4 percent severe. Conclusions: The whole presented adverse events, hematological alterations and reported events for drugs included in chemotherapy predominated. Adverse reactions classified as possible were identified, moderate and severe predominated(AU)


Subject(s)
Humans , Infant , Child, Preschool , Child , Drug-Related Side Effects and Adverse Reactions , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Remission Induction/methods , Epidemiology, Descriptive , Cross-Sectional Studies
2.
Arch. argent. pediatr ; 119(3): e242-e246, Junio 2021. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1248200

ABSTRACT

La leucemia linfoblástica aguda (LLA) es la patología oncológica más frecuente en pediatría, y corresponde al 23% de las neoplasias en menores de 15 años. Alrededor del 20% de los pacientes con LLA presentan recaídas, en la mayoría de los casos, en la médula ósea. Las recaídas extramedulares son inusuales y las dos localizaciones más frecuentes son el sistema nervioso central (SNC) y los testículos. Cuando las recaídas ocurren en el SNC, suelen manifestarse con un síndrome meníngeo. El síndrome hipotalámico se define como la presencia de hiperfagia, obesidad y/o cambios en el estado de ánimo, y es una forma de presentación clínica inusual de las recaídas en el SNC y debe alertar al pediatra para mantener un alto índice de sospecha.Se describen cuatro casos que se presentaron con síndrome hipotalámico al momento de desarrollar una recaída de LLA en el SNC


Acute lymphoblastic leukemia (ALL) is the most common malignancy in childhood, corresponding to 23% of cancer in children younger than 15 years old. About 20% of ALL cases will relapse, commonly in the bone marrow. Extramedullar relapses are unusual, and the two most frequent locations are CNS and testicles. ALL relapses, when diagnosed in the CNS, frequently present with clinical features of a meningeal syndrome. The hypothalamic syndrome, consisting of hyperphagia, obesity and / or behavior disturbances, corresponds to an unusual presentation of relapses in this location and should alert pediatricians to suspect it.We describe 4 ALL cases of hypothalamic syndrome at the time of CNS relapse


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Lymphoma, B-Cell , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnosis , Recurrence , Fatal Outcome , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Hypothalamic Diseases/diagnosis
3.
Article in Chinese | WPRIM | ID: wpr-880128

ABSTRACT

B-cell acute lymphoblastic leukemia (B-ALL) is a common malignant tumor in hematopoietic system. Although the remission rate of the patients with adult B-ALL is similar to those with childhood B-ALL, the rate of long-term disease-free survival (DFS) rate is significantly lower, once recurrence, the remission rate of routine chemotherapy is low and the prognosis is so poor. Based on the expression of tumor cell surface antigens(such as CD19, CD20 and CD22), the specific monoclonal antibodies, bispecific antibodies and chimeric antigen receptor T cells (CAR-T), and other targeted immunotherapy can greatly improve the efficacy of B-ALL patients, especially for patients with relapse and refractory. In this review, the progress of immunotherapy against B-ALL cell surface antigen is summarized briefly.


Subject(s)
Adult , Antigens, CD19 , Antigens, Surface , B-Lymphocytes , Burkitt Lymphoma , Child , Humans , Immunotherapy, Adoptive , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Receptors, Antigen, T-Cell
4.
Article in Chinese | WPRIM | ID: wpr-880028

ABSTRACT

OBJECTIVE@#To analyze the outcomes of the children suffered from philadelphia chromosome positive acute lymphoblastic leukemia (Ph@*METHODS@#21 cases of firstly diagnosed Ph@*RESULTS@#Among 21 patients, 17 were male and 4 were female with a median age of 8 years old (range, 4-12 years), the median follow-up time was 30 moths (range, 10-133 months). All the patients were treated with chemotherapy induced by the high-risk project of CCLG-ALL 2008. Among 14 patients treated with TKI plus chemotherapy, nine patients achieved complete remission. During 3 months after treatment, patients without complete molecular response or with the second complete remission and intensity desire of transplantation were treated with allo-HSCT, among 9 patients with allo-HSCT, six patients achieved long term survival.@*CONCLUSION@#At TKI era, TKI combined with strong chemotherapy can make Ph


Subject(s)
Aged , Child , Female , Hematopoietic Stem Cell Transplantation , Humans , Infant , Male , Philadelphia Chromosome , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Protein Kinase Inhibitors , Retrospective Studies
5.
Article in Chinese | WPRIM | ID: wpr-879584

ABSTRACT

OBJECTIVE@#To detect fusion gene with pathological significance in a patient with refractory and relapsed acute B cell lymphoblastic leukemia (B-ALL) and to explore its laboratory and clinical characteristics.@*METHODS@#Transcriptome sequencing was used to detect potential fusion transcripts. Other laboratory results and clinical data of the patient were also analyzed.@*RESULTS@#The patient was found to harbor TCF3 exon 17-ZNF384 exon 7 in-frame fusion transcript. The minimal residual disease (MRD) has remained positive after multiple chemotherapy protocols including CD19-, CD22- targeted chimeric antigen receptor T cells immunotherapy. The patient eventually achieved complete remission and sustained MRD negativity after allogeneic hemopoietic stem cell transplantation (allo-HSCT).@*CONCLUSION@#Transcriptome sequencing can effectively detect potential fusion genes with clinical significance in leukemia. TCF3-ZNF384 positive B-ALL has unique laboratory and clinical characteristics, may not well respond to chemotherapy and immunotherapy, and is more likely to relapse. Timely allo-HSCT treatment may help such patients to achieve long-term disease-free survival. TCF3-ZNF384 positive B-ALL is not uncommon in pediatric patients but has not been effectively identified.


Subject(s)
B-Lymphocytes , Basic Helix-Loop-Helix Transcription Factors/genetics , Child , Hematopoietic Stem Cell Transplantation , Humans , Laboratories , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Trans-Activators/genetics , Transcriptome
6.
Rev. cuba. hematol. inmunol. hemoter ; 35(1): e938, ene.-mar. 2019. graf
Article in Spanish | LILACS, CUMED | ID: biblio-1042892

ABSTRACT

La L-asparaginasa es un medicamento utilizado en distintas fases de todos los protocolos de tratamiento actuales de la leucemia linfoide aguda (LLA). Se describen múltiples manifestaciones secundarias a la L asparaginasa entre las que las reacciones alérgicas son las más frecuente. Se estudiaron 144 niños con diagnóstico de LLA tratados en el Instituto de Hematología e Inmunología, entre 1998 y el 2013. En 30 pacientes (21 por ciento) se presentaron reacciones alérgicas, similar a lo descrito en la literatura. El 76,6 por ciento de ellos habían recibido una dosis acumulativa menor de 80 000 UI (media de 48 757) y el mayor número de las reacciones alérgicas (86,7 por ciento) se reportó entre las dosis 9 y 18 recibidas (media de 11 dosis). Se observó una mayor supervivencia en los enfermos que recibieron más dosis (19 - 26 dosis) (p = 0.003). La sobrevida libre de eventos fue también mayor en este grupo (p= 0.357)(AU)


ABSTRACT L-asparaginase is a medication used in different phases of all current treatment protocols for acute lymphoid leukemia. Multiple secondary manifestations to L- asparaginase are described, and allergic reactions are the most frequent. We studied 144 children with acute lymphoblastic leukemia treated at the Instituto de Hematología e Inmunología between 1998 and 2013. Thirty patients (21 percent) had allergic reactions, similar to what is described in literature; 76.6 percent of them had received a cumulative dose of less than 80 000 IU (average of 48 757); and the highest number of allergic reactions (86.7 percent) was reported between doses 9 and 18 received (mean of 11 doses). A greater global survival was observed in patients who received more doses (19 - 26 doses) (p=0.003). Event free survival was also higher in this group (p= 0.357)(AU)


Subject(s)
Asparagine/adverse effects , Asparagine/therapeutic use , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Hypersensitivity/prevention & control
7.
Rev. cuba. hematol. inmunol. hemoter ; 33(4): 92-96, oct.-dic. 2017. ilus
Article in Spanish | LILACS | ID: biblio-960441

ABSTRACT

Ante una indicación de trasplante de células progenitoras hematopoyéticas se realiza la tipificación de los antígenos HLA de clase I y clase II al receptor y sus posibles donantes. En el departamento de Histocompatibilidad del Instituto de Hematología e Inmunología de La Habana se realizó un estudio familiar de histocompatibilidad a una paciente diagnosticada de leucemia linfoide aguda Ph+. La paciente y el padre presentaron el haplotipo HLA-A*03:01 B*39:10 C*12:03 DRB1*15:03 DQB1*06:02, que se identificó en el abuelo paterno, aunque por técnicas de baja resolución. A su vez, en el hermano y la madre también se tipificó un haplotipo compuesto por estos mismos alelos HLA-A, B, C, DRB1 y DQB1; y que se detectó en baja resolución en el abuelo materno. Sorprendentemente la paciente era HLA idéntica a la madre, cuando se esperaría que solo compartieran la mitad de los genes HLA. El hecho de que el haplotipo objeto de estudio apareciera en ambos padres de la paciente, quienes provenían de familias sin vínculos de parentesco conocido en al menos dos generaciones pasadas, puede considerarse un evento poco probable. Las investigaciones inmunogenéticas que están basadas en la tipificación HLA, no solo contribuyen a la selección de la mejor pareja donante receptor, sino que permiten a caracterizar el patrimonio genético del país(AU)


When a hematopoietic stem cell transplantation is indicated, the HLA class I and class II antigens are typed in the recipient and its possible donors. In the Histocompatibility department of the Institute of Hematology and Immunology of Havana, a family-based histocompatibility study was performed to a patient diagnosed with Ph+ acute lymphoid leukemia. The patient and the father presented the haplotype HLA-A*03:01 B*39:10 C*12:03 DRB1*15:03 DQB1*06:02, which was also identified in the paternal grandfather by low resolution techniques. In turn, a haplotype, composed of the same HLA-A, B, C, DRB1 and DQB1 alleles, was typed in the mother and the sibling and it was detected in low resolution in the maternal grandfather. Surprisingly, the patient was HLA identical to the mother, when they would be expected to share only half of the HLA genes. The fact that the haplotype under study appeared in both parents of the patient, who came from families without known ties of kinship in at least two past generations, can be considered an unlikely event. Immunogenetic investigations based on HLA typing, not only contribute to the selection of the best recipient donor pair, but also allow characterizing the nation's genetic heritage(AU)


Subject(s)
Humans , Male , Female , Family Characteristics/history , Hematopoietic Stem Cell Transplantation/methods , Histocompatibility Antigen H-2D/therapeutic use , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Medical History Taking/methods
8.
Braz. j. med. biol. res ; 50(1): e5426, 2017. tab, graf
Article in English | LILACS | ID: biblio-839242

ABSTRACT

IGH gene rearrangement and IGK-Kde gene deletion can be used as molecular markers for the assessment of B lineage acute lymphoblastic leukemia (B-ALL). Minimal residual disease detected based on those markers is currently the most reliable prognosis factor in B-ALL. The aim of this study was to use clonal IGH/IGK-Kde gene rearrangements to confirm B-ALL diagnosis and to evaluate the treatment outcome of Tunisian leukemic patients by monitoring the minimal residual disease (MRD) after induction chemotherapy. Seventeen consecutive newly diagnosed B-ALL patients were investigated by multiplex PCR assay and real time quantitative PCR according to BIOMED 2 conditions. The vast majority of clonal VH-JH rearrangements included VH3 gene. For IGK deletion, clonal VK1f/6-Kde recombinations were mainly identified. These rearrangements were quantified to follow-up seven B-ALL after induction using patient-specific ASO. Four patients had an undetectable level of MRD with a sensitivity of up to 10-5. This molecular approach allowed identification of prognosis risk group and adequate therapeutic decision. The IGK-Kde and IGH gene rearrangements might be used for diagnosis and MRD monitoring of B-ALL, introduced for the first time in Tunisian laboratories.


Subject(s)
Humans , Male , Female , Child, Preschool , Adolescent , Middle Aged , Biomarkers, Tumor/genetics , Gene Rearrangement/genetics , Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics , Precursor Cell Lymphoblastic Leukemia-Lymphoma/pathology , Neoplasm, Residual , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Real-Time Polymerase Chain Reaction , Sensitivity and Specificity
10.
Psicol. reflex. crit ; 28(3): 565-573, Jul-Sep/2015. tab
Article in Portuguese | INDEXPSI, LILACS | ID: lil-751998

ABSTRACT

Na atualidade cresce a preocupação com a neurotoxicidade do tratamento antineoplásico e o neurodesenvolvimento. O objetivo deste estudo foi comparar o impacto da modalidade de tratamento sobre a capacidade intelectiva de 22 sobreviventes de Tumores de Fossa Posterior e Leucemia Linfóide Aguda com idades entre seis e 14 anos. Participantes com astrocitoma foram submetidos à cirurgia; aqueles com meduloblastoma à cirurgia, à quimioterapia sistêmica e à radioterapia de crânio e neuroeixo (54Gy) e; aqueles com LLA à quimioterapia sistêmica e intratecal. Apenas os participantes com astrocitoma obtiveram desempenho dentro do esperado. Observou-se contrastes estatisticamente significativos entre os grupos, notadamente entre as crianças com meduloblastoma e as demais nos escores não verbais. Sugere-se que a combinação cirurgia, quimioterapia sistêmica e radioterapia potencializou as sequelas cognitivas, e reforça-se a hipótese de que a radioterapia acarreta danos à substância branca. A quimioterapia intratecal associada à sistêmica promoveu impactos significativos sobre o funcionamento executivo.


Concerns about the neurotoxicity of antineoplastic treatment and neurodevelopment are increasing nowadays. The aim of this study was to compare the impact of treatment modality on intellectual functioning of 22 survivors of Posterior Fossa Tumors and Acute Lymphoblastic Leukemia aged from six to 14 years. The astrocytoma group underwent surgery; the medulloblastoma group underwent surgery, systemic chemotherapy, and cranial and neuraxis radiation (54Gy); the LLA group underwent systemic and intrathecal chemotherapy. Only the astrocytoma group obtained average performance. Significant contrasts were obtained between groups, especially among the medulloblastoma group and others in non-verbal scores. Results suggest that the combination of surgery, radiotherapy and systemic chemotherapy increase the cognitive sequelae and enhance the hypothesis that radiation damages white matter. The association between intrathecal and systemic chemotherapy leads to significant impact on executive functioning.


Subject(s)
Humans , Male , Female , Child , Adolescent , Radiotherapy/adverse effects , Astrocytoma/therapy , Cognition , Drug Therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Medulloblastoma/therapy
11.
Rev. gaúch. enferm ; 36(2): 76-81, Apr-Jun/2015.
Article in English | LILACS, BDENF | ID: lil-752576

ABSTRACT

OBJECTIVE: To understand the influence of play in the care process as perceived by children with cancer. METHOD: A descriptive, exploratory and qualitative study conducted in a children's cancer unit in Natal, Rio Grande do Norte, Brazil. Data were collected between October 2013 and January 2014 by means of photographic records and semi-structured interviews with eight children, and content analysis with emphasis on two categories: Auxiliary instruments during play; and The influence of play in the process of care. RESULTS: Recreational activities involve watching television, using computers, games and toys, drawing, the playroom and the clown, which provide fun, feelings of joy, distraction and interaction with other people. CONCLUSION: There are several activities at the hospital that are considered play-related and, for the children, they all benefit their care process. .


OBJETIVO: Comprender la influencia de lo lúdico en el proceso de atención, en la percepción de los niños con cáncer. MÉTODO: Estudio cualitativo, exploratorio descriptivo, realizado en un sector de oncología pediátrica en Natal, Rio Grande do Norte, Brasil. Los datos fueron recogidos entre los meses de octubre de 2013 y enero de 2014, a través de los registros fotográficos y entrevistas semiestructuradas con ocho hijos, y analizados según el análisis de contenido, destacando dos categorías de discusión: Los instrumentos auxiliares en la alegría; La influencia de lo lúdico en el proceso de atención. RESULTADOS: Las actividades recreativas implican ver televisión, usar computadoras, juegos y juguetes, la realización de dibujos y el payaso, que proporcionan diversión, sentimientos de alegría, distracción y la interacción con los demás. CONCLUSIÓN: Hay varias actividades, en el hospital, entendido como lúdico y que, para el niño, todos proporcionan beneficios para su proceso de atención. .


OBJETIVOS: Compreender a influência do lúdico para o processo de cuidar, na percepção de crianças com câncer. MÉTODO: Estudo qualitativo, exploratório descritivo, realizado em um setor de oncopediatria em Natal, Rio Grande do Norte, Brasil. Os dados foram coletados entre os meses de outubro de 2013 e janeiro de 2014, por meio de registros fotográficos e entrevista semiestruturada, com oito crianças, e analisados conforme a Análise de Conteúdo, destacando-se duas categorias de discussão: Os instrumentos auxiliares na ludicidade; e A influência do lúdico no processo de cuidar. RESULTADOS: As atividades lúdicas envolvem o assistir à televisão, o uso de computadores, os jogos e os brinquedos, a realização de desenhos, a brinquedoteca e o palhaço, os quais proporcionam diversão, sentimentos de alegria, distração e interação com outras pessoas. CONCLUSÃO: Existem diversas atividades, no hospital, entendidas como lúdicas, todas as quais, para a criança, proporcionam benefícios para o seu processo de cuidar. .


Subject(s)
Humans , Male , Female , Child , Child, Hospitalized/psychology , Neoplasms/therapy , Play Therapy , Cerebellar Neoplasms/psychology , Cerebellar Neoplasms/therapy , Interviews as Topic , Medulloblastoma/nursing , Medulloblastoma/psychology , Medulloblastoma/therapy , Nurse-Patient Relations , Nursing Process , Neoplasms/nursing , Neoplasms/psychology , Patient Acceptance of Health Care , Play and Playthings , Play Therapy/instrumentation , Play Therapy/methods , Precursor Cell Lymphoblastic Leukemia-Lymphoma/nursing , Precursor Cell Lymphoblastic Leukemia-Lymphoma/psychology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Qualitative Research , Video Games
12.
Rev. cuba. hematol. inmunol. hemoter ; 30(1): 36-46, ene.-mar. 2014.
Article in Spanish | LILACS | ID: lil-705662

ABSTRACT

Introducción: la leucemia linfoide aguda (LLA) es la enfermedad maligna más frecuente en la infancia y la primera que se trató con un protocolo común en Cuba. Se han aplicado diferentes protocolos a lo largo del tiempo y actualmente existen en el país 10 centros que tratan niños con LLA. Objetivo: presentar los resultados alcanzados en el tratamiento de la LLA desde 2002 hasta 2008 con el protocolo del grupo ALLIC (Acute Lymphoblastic Leukemia Intercontinental). Métodos: se trataron 166 niños menores de 18 años al inicio de la enfermedad. Para conformar los grupos pronóstico se utilizaron diferentes criterios que incluyeron la edad y el número de leucocitos en el momento del diagnóstico, las alteraciones moleculares y la respuesta al tratamiento. Resultados: la supervivencia libre de eventos (SLE) a los 4 años fue del 69 por ciento y la supervivencia global del 78 por ciento. La SLE en los diferentes grupos pronósticos fue del 85 por ciento para los pacientes de riesgo estándar, 77 por ciento para los de riesgo intermedio y 59 por ciento para los de riesgo alto. El porcentaje de remisión inicial fue inferior al obtenido por el grupo total. La mayoría de las muertes ocurrieron al inicio de la aplicación del protocolo. La recaída hematológica fue la causa más frecuente de terminación de la remisión completa. Las recaídas del sistema nervioso central, las testiculares y las combinadas fueron inferiores al 5 por ciento. La presencia de reordenamientos genéticos del tipo bcr/abl o MLL/AF4 se confirmaron como elementos de muy mal pronóstico. Conclusiones: estos resultados, aunque son susceptibles de ser mejorados, muestran un nivel adecuado, sobre todo si se tiene en cuenta que se han logrado en un país en vías de desarrollo


Introduction: Acute lymphoid leukaemia (ALL) is the most frequent malignant disease in childhood and the first one to be treated with a common protocol in Cuba. Different protocols have been used and at present there are 10 health centers in Cuba treating children with ALL. Objective: To present the results achieved in the treatment of ALL from 2002 to 2008 with protocol ALLIC (Acute Lymphoblastic Leukemia Intercontinental). Methods: 166 children under 18 years old at the beginning of the disease were treated. Different criteria were used to make prognostic groups which included age and leukocyte counts in the peripheral smear at diagnosis, DNA molecular rearrangements and response to therapy. Results: Event free survival (EFS) after 4 years for the whole group was 69 percent and overall survival (SV) was 78 percent. EFS in the different prognostic groups were 85 percent for standard risk patients, 77 percent for the intermediate risk group and 59 percent for high risk children. Percentage of initial remission in our patients was lower than the one obtained for the whole group. The majority of early deaths occurred at the beginning of the protocol application. Bone marrow relapses were the more frequent ones. Central nervous system, testicular or combined relapses were lower than 5 percent. DNA rearrangements for bcr/ abl or MLL/AF4 were signs of very bad prognosis. Conclusions: These results, even when susceptible to be better, show an adequate level considering that they were achieved in a developing country


Subject(s)
Humans , Child , Antineoplastic Combined Chemotherapy Protocols , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnosis , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Antineoplastic Protocols/standards , Disease-Free Survival
15.
Rev. cuba. hematol. inmunol. hemoter ; 27(3): 283-290, jul.-set. 2011.
Article in Spanish | LILACS | ID: lil-615356

ABSTRACT

Las enzimas de biotransformación y eliminación de los fármacos en pacientes con leucemia linfoide aguda tienen una acción determinante en el efecto terapéutico de los medicamentos antineoplßsicos. La presencia y actividad de estos complejos enzimáticos está codificada genéticamente y sujeta a variaciones alélicas, cuyas frecuencias son variables en las diferentes poblaciones humanas. Este polimorfismo genético influye sobre la efectividad terapéutica de los medicamentos y condiciona la carencia de toxicidad o presencia de esta, que en ocasiones puede ser fatal. Las enzimas tiopurin-metil-transferasa, metilén-tetrahidrofolato-reductasa y glutatión-tranferasa son sistemas destoxificadores de algunos de los quimioterápicos empleados en el tratamiento de la leucemia linfoide aguda. En este trabajo se revisan las características genéticas de estas enzimas, la frecuencia de sus polimorfismos y las implicaciones clínicas de su expresión. De igual modo se discute la importancia y los beneficios del genotipaje previo al inicio del tratamiento, con el fin de modificar las dosis de los medicamentos para optimizar su efecto terapéutico y disminuir su toxicidad. La farmacogenética constituye un área de creciente interés que ha tenido un desarrollo considerable en los últimos años, su conocimiento e implementación nos colocará en el camino de la medicina personalizada


The biotransformation and elimination enzymes of drugs in patients suffering from acute lymphoid leukemia play a decisive role on the therapeutical effect of anti-neoplastic drugs. The presence and activity of these enzymatic complexes are genetically coded and subjected to allele variations, the frequency of which is variable in the different human populations. This genetic polymorphism has an impact on the therapeutic effectiveness of drugs and determines the lack or the existence of toxicity that may sometimes become lethal. The enzymes called thiopurine-methyltransferase, methylen-tetrahydropholate-reductase and glutathione-transferase are detoxifying systems of some of the chemotherapeutic drugs that are used for the treatment of acute lymphoid leukemia. This paper reviewed the genetic characteristics of the enzymes, the frequency of polymorphisms and the clinical implications of their expression. Similarly, the importance and the benefits of genotyping before the treatment were discussed in order to change the drug doses to maximize the therapeutic effect and reduce toxicity. Pharmacogenetics has experienced great development in the last few years and draws growing interest; the knowledge about and the implementation of this discipline will take us to the customized medicine


Subject(s)
Pharmacogenetics/methods , Precursor Cell Lymphoblastic Leukemia-Lymphoma/enzymology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Genotyping Techniques/methods
17.
Rev. bras. ginecol. obstet ; 33(8): 174-181, ago. 2011. tab
Article in Portuguese | LILACS | ID: lil-608241

ABSTRACT

RESUMO OBJETIVO: Descrever as complicações maternas e os resultados perinatais entre as gestantes com diagnóstico de leucemia que foram acompanhadas no pré-natal e no parto em hospital universitário. MÉTODOS: Estudo retrospectivo do período de 2001 a 2011, que incluiu 16 gestantes portadoras de leucemia acompanhadas pela equipe de pré-natal especializado em hemopatias e gestação. Nas leucoses agudas, diagnosticadas após o primeiro trimestre, a recomendação foi realizar a quimioterapia apesar da gestação em curso. Nas gestantes com leucoses crônicas, quando controladas do ponto de vista hematológico, foram mantidas sem medicação durante a gravidez, ou, foi introduzida terapêutica antineoplásica após o primeiro trimestre. Foram analisadas as complicações maternas e os resultados perinatais. RESULTADOS: A leucemia linfoide aguda (LLA) foi diagnosticada em cinco casos (31,3 por cento), a leucemia mieloide aguda (LMA) em dois casos (12,5 por cento) e a leucemia mieloide crônica (LMC) em nove casos (56,3 por cento). Nos casos de leucemias agudas, dois (28,6 por cento) casos foram diagnosticados no primeiro trimestre, dois (28,6 por cento) no segundo e três (42,9 por cento) no terceiro. Duas gestantes com LLA diagnosticada no primeiro trimestre optaram pelo aborto terapêutico. Quatro casos de leucemia aguda receberam tratamento quimioterápico na gestação, com diagnóstico estabelecido após a 20ª semana. Em um caso de LLA com diagnóstico tardio (30ª semana) a quimioterapia foi iniciada após o parto. Todas as gestantes com leucemia aguda evoluíram com anemia e plaquetopenia, quatro casos (57,1 por cento) evoluíram com neutropenia febril. Das gestantes com LMC, quatro utilizavam mesilato de imatinibe quando engravidaram, três delas suspenderam no primeiro trimestre e uma no segundo. Durante a gravidez, três (33,3 por cento) não necessitaram de terapêutica antineoplásica após suspensão do imatinibe; e em seis (66,7 por cento) foram utilizadas as seguintes drogas: interferon (n=5) e/ou hidroxiureia (n=3). No grupo de gestantes com LMC, verificou-se a ocorrência de anemia em quatro casos (44,4 por cento) e plaquetopenia em um (11,1 por cento). Quanto aos resultados perinatais, nas gestações complicadas pela leucemia aguda, a média da idade gestacional no parto foi de 32 semanas (desvio padrão - DP=4,4) e a média do peso do recém-nascido foi 1476 g (DP=657 g). Houve 2 (40,0 por cento) óbitos perinatais (um fetal e um neonatal). Nas gestações complicadas pela LMC, a média da idade gestacional no parto foi de 37,6 semanas (DP=1,1) e a média do peso do recém-nascido foi 2870 g (DP=516 g); não houve morte perinatal e nenhuma anomalia fetal foi detectada. CONCLUSÕES: É elevada a morbidade materna e fetal nas gestações complicadas pela leucemia aguda; enquanto que, nas complicadas pela LMC, o prognóstico materno e fetal parece ser mais favorável, com maior facilidade no manejo das complicações.


PURPOSE: To describe the maternal and perinatal outcomes of pregnant women diagnosed with leukemia who were followed up for prenatal care and delivery at a university hospital. METHODS: A retrospective study of the period from 2001 to 2011, which included 16 pregnant women with a diagnosis of leukemia followed by antenatal care specialists in hematological diseases and pregnancy. For acute leukemia diagnosed after the first trimester, the recommendation was to perform chemotherapy despite the current pregnancy. For chronic leukemia, patients who were controlled in hematological terms were maintained without medication during pregnancy, or chemotherapy was introduced after the first trimester. We analyzed the maternal and perinatal outcome. RESULTS: Acute lymphoblastic leukemia (ALL) was diagnosed in five cases (31.3 percent), acute myeloid leukemia (AML) in two cases (12.5 percent) and chronic myeloid leukemia (CML) in nine cases (56.3 percent). Of the cases of acute leukemia, two (28.6 percent) were diagnosed in the first trimester, two (28.6 percent) in the second and three (42.9 percent) in the third. Two patients with ALL diagnosed in the first trimester opted for therapeutic abortion. Four patients with acute leukemia received chemotherapy during pregnancy, with a diagnosis established after the 20th week. In one case of ALL with a late diagnosis (30 weeks), chemotherapy was started after delivery. All pregnant women with acute leukemia developed anemia and thrombocytopenia, and four (57.1 percent) developed febrile neutropenia. Of nine pregnant women with CML, four were treated with imatinib mesylate when they became pregnant, with treatment being interrupted in the first trimester in three of them and in the second trimester in one. During pregnancy, three patients (33.3 percent) required no chemotherapy after discontinuation of imatinib, and six (66.7 percent) were treated with the following drugs: interferon (n=5) and/or hydroxyurea (n=3 ). In the group of pregnant women with CML, anemia occurred in four (44.4 percent) cases and thrombocytopenia in one (11.1 percent). The perinatal outcomes of pregnancies complicated by acute leukemia were as follows: mean gestational age at delivery was 32 weeks (standard deviation - SD=4.4) and the mean birth weight was 1476 g (SD=657 g), there were 2 (40.0 percent) perinatal deaths (a fetal one and a neonatal one). In pregnancies complicated by CML, the mean gestational age at delivery was 37.6 weeks (SD=1.1) and the mean birth weight was 2870 g (SD=516 g). There was no perinatal death and no fetal abnormality was detected. CONCLUSIONS: Maternal and fetal morbidity is high in pregnancies complicated by acute leukemia. Whereas, in pregnancies complicated by CML, the maternal and fetal prognosis appears to be more favorable, with greater ease in management of complications.


Subject(s)
Adolescent , Adult , Female , Humans , Infant, Newborn , Pregnancy , Young Adult , Leukemia, Myeloid/therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Pregnancy Complications, Neoplastic/therapy , Pregnancy Outcome , Retrospective Studies
18.
Indian J Cancer ; 2011 Jul-Sept; 48(3): 310-315
Article in English | IMSEAR | ID: sea-144487

ABSTRACT

Background: Pediatric acute lymphoblastic leukemia (ALL) is a biologically heterogeneous disease and socioeconomic and environmental factors are considered to be an important determinant of its immunophenotype. The aim of this analysis is to study the time trend in the immunophenotype of pediatric acute lymphoblastic leukemia (ALL) cases in our geographic setting. Materials and Methods: A total of 639 new pediatric ALL cases immunophenotyped during 1989-2009 forms the basis of this analysis. Representative bone marrow or peripheral blood of these patients was immunophenotyped flowcytometrically using an extensive panel of monoclonal antibodies. Results: During early phase of our study we noticed a relative excess of T-ALL and a paucity of common acute lymphoblastic leukemia (C-ALL) in contrast to western data. Over a period of 20 years we witnessed a gradual reduction in pediatric T-ALL cases and a proportionate increase in C-ALL cases. Conclusion: We find that this change of pattern is synchronizing with the socioeconomic and industrial development prevailing in our geographic setting and suggest a possible link between the predominant immunophenotype of pediatric ALL cases and the environmental and socioeconomic factors prevailing in that locality.


Subject(s)
Adolescent , Antibodies, Monoclonal/diagnosis , Child , Child, Preschool , Disease-Free Survival , Female , Flow Cytometry , Humans , Immunophenotyping/methods , Incidence , India/epidemiology , Male , Neprilysin/blood , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnosis , Precursor Cell Lymphoblastic Leukemia-Lymphoma/epidemiology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/epidemiology , Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/immunology
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