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1.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(4): 489-493, Oct.-Dec. 2021. tab
Article in English | LILACS | ID: biblio-1350813

ABSTRACT

ABSTRACT Objective: Low levels of neutrophils can be an intrinsic condition, with no clinical consequences or immunity impairment. This condition is the benign constitutional neutropenia (BCN), defined as an absolute neutrophils count (ANC) ≤2000 cells/mm. Diagnosis of BCN is of exclusion where patients are submitted to blood tests and possibly to invasive diagnostic search until secondary causes of neutropenia are ruled out. The natural history of the disease suggests benign evolution and Brazilian study showed an overall frequency of 2.59%. The main mechanisms include reduced neutrophil production, increased marginalization, extravasation to the tissues and immune destruction. Genetic studies showed strong association between the single nucleotide variant rs2814778 located on chromosome 1q23.2 in the promoter region of the atypical chemokine receptor 1 (Duffy blood group system) gene (ACKR1, also termed DARC) and BCN. The aim of this study is to evaluate FY phenotypes and genotypes including the analysis of the rs2814778 SNP in Brazilian patients with BCN in order to determine an effective diagnostic tool, allowing reassurance of the patient and cost reduction in their care. Methods: Case control study, with 94 individuals (18 patients and 76 controls). Phenotyping was performed by gel test and genotyping was performed by PCR-RFLP. Results: White blood cell (WBC) and absolute neutrophils (AN) counts showed lower levels in patients compared to controls. In the patient group 83.3% were genotyped as FY*B/FY*B. The SNP rs2814778 (-67T > C) was identified in 77.8% of the patients genotyped as FY*B-67C/FY*B-67C. In the control group, 72.7% were homozygous for the wild type and 23.3% were heterozygous. Conclusion: This study reinforces that FY phenotyping and genotyping can be used to detect most people with BCN, avoiding excessive diagnostic investigation. Besides, this procedure may reduce health costs and be reproductible in clinical practice.


Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Middle Aged , Aged , Duffy Blood-Group System , Genotyping Techniques , Neutropenia , Immunophenotyping , Diagnostic Tests, Routine , Neutrophils
2.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(1): 65-86, Jan.-Mar. 2021. tab
Article in English | LILACS | ID: biblio-1154293

ABSTRACT

ABSTRACT Autoimmune diseases are an important field for the development of bone marrow transplantation, or hematopoietic stem cell transplantation. In Europe alone, almost 3000 procedures have been registered so far. The Brazilian Society for Bone Marrow Transplantation (Sociedade Brasileira de Transplantes de Medula Óssea) organized consensus meetings for the Autoimmune Diseases Group, to review the available literature on hematopoietic stem cell transplantation for autoimmune diseases, aiming to gather data that support the procedure for these patients. Three autoimmune diseases for which there are evidence-based indications for hematopoietic stem cell transplantation are multiple sclerosis, systemic sclerosis and Crohn's disease. The professional stem cell transplant societies in America, Europe and Brazil (Sociedade Brasileira de Transplantes de Medula Óssea) currently consider hematopoietic stem cell transplantation as a therapeutic modality for these three autoimmune diseases. This article reviews the evidence available.


Subject(s)
Humans , Scleroderma, Systemic , Crohn Disease , Bone Marrow Transplantation , Hematopoietic Stem Cell Transplantation , Scleroderma, Diffuse , Multiple Sclerosis
3.
Arq. gastroenterol ; 57(3): 278-282, July-Sept. 2020. tab, graf
Article in English | LILACS | ID: biblio-1131670

ABSTRACT

ABSTRACT BACKGROUND: Weight loss and body composition changes are common in patients with pancreatic cancer. Computed tomography (CT) images are helpful to investigate body composition and its changes and to discriminate the different kinds of body tissues. Patients with pancreatic cancer routinely undergo CT scans. OBJECTIVE: To verify the association of muscle mass and visceral fat measured by CT with toxicity and survival of patients with pancreatic cancer. METHODS: We evaluated the imaging of the abdomen of all consecutive adult patients with pancreatic cancer treated between October 2007 and September 2015 in our service, to assess skeletal muscle mass and fat, intramuscular fat and visceral fat. We graded treatment toxicity symptoms according to the Common Toxicity Criteria of the United States National Cancer Institute (version 2.0). RESULTS: The study involved 17 patients, with a mean age of 63 (±10) years (range: 51-73 years). Eleven (65%) were male. The mean initial body mass index (BMI) was 26 kg/m2 (±3) and 23 kg/m2 (±3) after treatment. The mean weight loss was 10.0 kg (±6.8; 13%). Sarcopenia was present in 47% of patients, and it was not associated with significant differences in muscle mass, visceral fat, toxicity or survival. The mean skeletal muscle attenuation was 36 Hounsfield units, not associated with survival or treatment toxicity. Mean muscle mass was not associated with toxicity either. However, there was a significant inverse association between toxicity and visceral fat. CONCLUSION: Muscle mass had no impact on the survival or on treatment toxicity among the patients with pancreatic cancer. However, the visceral fat exerted a protective effect against the treatment toxicity. We stress the importance of further studies on visceral fat associated with prognosis and toxicity in cancer patients.


RESUMO CONTEXTO: Perda de peso e mudanças na composição corpórea são frequentes em pacientes com câncer do pâncreas. Imagens de tomografia computadorizada são úteis na investigação da composição corporal e suas mudanças e para discriminar diferentes tipos de tecidos corporais. Pacientes com câncer pancreático são submetidos à tomografia computadorizada rotineiramente. OBJETIVO: Verificar a associação entre massa muscular e gordura visceral medida por tomografia computadorizada e a toxicidade do tratamento e a sobrevida de pacientes com câncer de pâncreas. MÉTODOS: Avaliamos imagens tomográficas abdominais de todos os pacientes adultos com câncer pancreático consecutivamente tratados entre outubro de 2007 e setembro de 2015 no serviço, para estimativa da massa muscular esquelética e gordura, tecido adiposo intramuscular e visceral. Classificamos a toxicidade do tratamento conforme os Critérios Comuns de Toxicidade do Instituto Nacional do Câncer dos Estados Unidos (versão 2.0). RESULTADOS: O estudo envolveu 17 pacientes, com idade média de 63 (±10) anos (51-73 anos); 11 (65%) homens. O índice de massa corporal médio inicial foi de 26 kg/m2 (±3); 23 kg/m2 (±3) após o tratamento. A perda de peso média foi de 10,0 kg (±6,8; 13%). Observamos sarcopenia em 47% dos pacientes, não associada a diferenças significativas na massa muscular, tecido adiposo visceral, toxicidade ou sobrevivência. A atenuação média do músculo esquelético foi de 36 unidades Hounsfield, não associada à sobrevivência ou toxicidade. A massa muscular média também não foi associada à toxicidade. No entanto, observamos associação inversa significativa entre toxicidade e gordura visceral. CONCLUSÃO: A massa muscular não teve impacto na sobrevida ou na toxicidade do tratamento dos pacientes com câncer pancreático. No entanto, a gordura visceral exerceu efeito protetor contra a toxicidade. Ressaltamos a importância de mais estudos sobre a gordura visceral associada ao prognóstico e à toxicidade em pacientes com câncer.


Subject(s)
Humans , Male , Female , Aged , Pancreatic Neoplasms/diagnostic imaging , Prognosis , Body Composition , Body Mass Index , Pilot Projects , Cross-Sectional Studies , Retrospective Studies , Sarcopenia , Middle Aged
4.
Hematol., Transfus. Cell Ther. (Impr.) ; 42(3): 221-229, July-Sept. 2020. tab, graf
Article in English | LILACS | ID: biblio-1134038

ABSTRACT

ABSTRACT Background: Immunological life-threatening complications frequently occur in post-hematopoietic stem cell transplantation (HSCT), despite matching recipient and donor (R/D) pairs for classical human leukocyte antigens (HLA). Studies have shown that R/D non-HLA disparities within the major histocompatibility complex (MHC) are associated with adverse effects post-HSCT. Methods: We investigated the impact of mismatches of single-nucleotide polymorphisms (SNPs) in C4A/C4B genes, for showing the highest diversity in the MHC gamma block, on 238 patients who underwent HLA 10/10 unrelated donor (URD) HSCT. The endpoints were acute graft-versus-host disease (aGVHD), chronic graft-versus-host disease (cGVHD) and mortality. One hundred and twenty-nine R/D pairs had 23 C4-SNPs typed by PCR-SSP (Gamma-Type™v.1.0), and 109 R/D pairs had these 23 SNPs identified by next-generation sequencing (NGS) using the Illumina platform. Results: The percentage of patients who received HSC from HLA 10/10 donors with 1-7 mismatches was 42.9%. The R/D pairs were considered C4 mismatched when bearing at least one disparity. These mismatches were not found to be risk factors for aGVHD, cGVHD or mortality after unrelated HSCT when SNPs were analyzed together (matched or mm ≥ 1), independently or according to the percentage of incompatibilities (full match for 23 SNPs; 1-3 mm and >3 mm). An exception was the association between 1-3 mismatches at the composite of SNPs C13193/T14952/T19588 with the development of aGVHD (P = 0.012) and with grades III-IV of this disease (P = 0.004). Conclusion: Our data are not consistent with the hypothesis that disparities in C4A/C4B SNPs increase the risks of post-HSCT adverse effects for the endpoints investigated in this study.


Subject(s)
Humans , Child , Adolescent , Adult , Genes, MHC Class I , Complement C4a , Complement C4b , Hematopoietic Stem Cell Transplantation , Polymorphism, Single Nucleotide , Polymorphism, Genetic , Mortality , Graft vs Host Disease
5.
Einstein (Säo Paulo) ; 18: AE4530, 2020. tab, graf
Article in English | LILACS | ID: biblio-1056061

ABSTRACT

ABSTRACT The nutritional status of patients submitted to hematopoietic stem cell transplant is considered an independent risk factor, which may influence on quality of life and tolerance to the proposed treatment. The impairment of nutritional status during hematopoietic stem cell transplant occurs mainly due to the adverse effects resulting from conditioning to which the patient is subjected. Therefore, adequate nutritional evaluation and follow-up during hematopoietic stem cell transplant are essential. To emphasize the importance of nutritional status and body composition during treatment, as well as the main characteristics related to the nutritional assessment of the patient, the Brazilian Consensus on Nutrition in Hematopoietic Stem Cell Transplant: Adults was prepared, aiming to standardize and update Nutritional Therapy in this area. Dietitians, nutrition physicians and hematologists from 15 Brazilian centers thar are references in hematopoietic stem cell transplant took part.


RESUMO O estado nutricional do paciente submetido ao transplante de células-tronco hematopoéticas é considerado fator de risco independente, podendo influenciar na qualidade de vida e na tolerância ao tratamento proposto. O comprometimento do estado nutricional durante o transplante de células-tronco hematopoéticas ocorre principalmente devido aos efeitos adversos decorrentes do condicionamento ao qual o paciente é submetido. Desta forma, a adequada avaliação nutricional e o acompanhamento durante o transplante de células-tronco hematopoéticas tornam-se imprescindíveis. Com o objetivo de salientar a importância do estado nutricional e da composição corporal durante o tratamento, bem como as principais características relacionadas à avaliação nutricional do paciente, o Consenso Brasileiro de Nutrição em Transplante de Células-Tronco Hematopoiéticas: Adulto foi elaborado visando uniformizar e atualizar a Terapia Nutricional nesta área. Com a participação de nutricionistas, nutrólogos e hematologistas de 15 centros brasileiros referências em transplante de células-tronco hematopoéticas


Subject(s)
Humans , Adult , Nutritional Status , Hematopoietic Stem Cell Transplantation/standards , Nutrition Therapy/standards , Brazil , Nutrition Assessment , Anthropometry , Parenteral Nutrition/methods , Parenteral Nutrition/standards , Hematopoietic Stem Cell Transplantation/adverse effects , Transplantation Conditioning , Nutrition Therapy/methods
6.
Einstein (Säo Paulo) ; 18: eRC5111, 2020. graf
Article in English | LILACS | ID: biblio-1056048

ABSTRACT

ABSTRACT Situs inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.


RESUMO Situs inversus totalis é uma anormalidade congênita autossômica recessiva rara em que os órgãos mediastinais e abdominais encontram-se em posição espelhada em relação à topografia habitual. A literatura relata alguns casos de concomitância do situs inversus totalis com outras condições: anomalias espinhais, malformações cardíacas e doenças hematológicas, como púrpura trombocitopênica idiopática, que é uma doença autoimune com plaquetopenia, devido à destruição dos trombócitos ou supressão da sua produção. Esse artigo teve o objetivo de relatar coexistência de situs inversus totalis e púrpura trombocitopênica idiopática.


Subject(s)
Humans , Male , Young Adult , Situs Inversus/complications , Situs Inversus/diagnostic imaging , Purpura, Thrombocytopenic, Idiopathic/complications , Situs Inversus/pathology , Radiography, Panoramic , Tomography, X-Ray Computed
7.
Hematol., Transfus. Cell Ther. (Impr.) ; 41(1): 69-75, Jan.-Mar. 2019. tab, ilus
Article in English | LILACS | ID: biblio-1002052

ABSTRACT

Abstract Graft-versus-host disease is one of the main causes of morbidity and mortality in patients submitted to hematopoietic stem cell transplantation. This study reviewed the prevalence of lower female genital tract graft-versus-host disease following hematopoietic stem cell transplantation. A systematic search of the literature for articles published between 1982 and 2015 was performed. A growing number of young women suffering from malignant and benign hematological diseases are receiving allogeneic hematopoietic stem cell transplantation with very satisfactory results in relation to the disease itself. However, these patients face gynecological problems due to graft-versus-host disease. Correct diagnosis and early management are needed to avoid irreversible complications.


Subject(s)
Humans , Female , Hematopoietic Stem Cell Transplantation , Genital Neoplasms, Female , Genitalia, Female , Graft vs Host Disease
8.
Einstein (Säo Paulo) ; 17(3): eAE4510, 2019. tab
Article in English | LILACS | ID: biblio-1019800

ABSTRACT

ABSTRACT Objective To standardize the investigation and clinical management of women with laboratory and/or clinical abnormalities suggestive of thrombophilia, in order to optimize antithrombotic approach and indication of laboratory tests. Methodology A discussion was carried out among 107 physicians (gynecologists/obstetricians, hematologists and vascular surgeons) present at a forum held at the Hospital Israelita Albert Einstein, in São Paulo (SP), Brazil. As a minimum criterion, 80% agreement was established in the voting to each recommendation of conduct in the final document. The cases in which there was agreement below 80% were discussed again, reaching a consensual agreement of conduct for the document writing. Conclusion The standardization of an institutional consensus of suggestions of clinical approach contributes to a better management of the group to be evaluated and minimizes risks of intercurrent events. This was the first national consensus on the investigation of thrombophilia in women.


RESUMO Objetivo Padronizar a investigação e o manejo clínico de mulheres com anormalidades clínicas e exames laboratoriais sugestivos de trombofilia, para melhorar a abordagem antitrombótica e otimizar a indicação de exames laboratoriais. Metodologia Foi conduzida discussão incluindo 107 médicos (ginecologistas/obstetras, hematologistas e cirurgiões vasculares) participantes de um fórum realizado no Hospital Israelita Albert Einstein, em São Paulo (SP). Como critério mínimo, estabeleceu-se concordância de 80% em votação para cada recomendação de conduta registrada em documento como diretrizes finais. Os casos em que a concordância esteve abaixo de 80% foram rediscutidos, para definir consenso na conduta. Conclusão A padronização e o estabelecimento de consenso institucional, com sugestões para abordagem clínica, contribui para melhorar o manejo do grupo a ser avaliado e minimizar os riscos de intercorrências. Este foi o primeiro consenso nacional sobre investigação de trombofilia em mulheres.


Subject(s)
Humans , Female , Pregnancy , Thrombophilia/diagnosis , Thrombophilia/etiology , Thrombophilia/drug therapy , Brazil , Mass Screening , Consensus
9.
Hematol., Transfus. Cell Ther. (Impr.) ; 40(4): 317-325, Oct.-Dec. 2018. tab, graf
Article in English | LILACS | ID: biblio-984501

ABSTRACT

ABSTRACT Background: Although chronic lymphocytic leukemia is basically a B cell disease, its pathophysiology and evolution are thought to be significantly influenced by T cells, as these are probably the most important interaction partner of neoplastic B cells, participating in their expansion, differentiation and survival. Chronic lymphocytic leukemia B cells may also drive functional and phenotypic changes of non-malignant T cells. There are few data about the association between memory T cells and prognosis, especially related to ZAP-70, a common reliable surrogate of the gold standard chronic lymphocytic leukemia prognostic markers. Objective: The aim of this study was to investigate whether the expression of ZAP-70 in chronic lymphocytic leukemia patients is associated with abnormal patterns of the distribution of naïve and memory T cells related to crosstalk between these cells. Methods: In this cross-sectional, controlled study, patients with chronic lymphocytic leukemia were compared with healthy blood donors regarding the expression of ZAP-70 and the distribution of naïve and memory T cell subsets in peripheral blood as measured by flow cytometry. Results: ZAP-70 positive patients presented an increased frequency and absolute number of central memory CD4+ T cells, but not CD8+ T cells, compared to ZAP-70 negative patients and age-matched apparently healthy donors. Conclusions: Because central memory CD4+ T cells are located in lymph nodes and express CD40L, we consider that malignant ZAP-70-positive B cells may receive beneficial signals from central memory CD4+ T cells as they accumulate, which could contribute to more aggressive disease.


Subject(s)
Humans , Male , Female , Protein-Tyrosine Kinases , T-Lymphocytes , Leukemia, Lymphocytic, Chronic, B-Cell , ZAP-70 Protein-Tyrosine Kinase
10.
Einstein (Säo Paulo) ; 15(4): 492-495, Oct.-Dec. 2017. tab, graf
Article in English | LILACS | ID: biblio-891420

ABSTRACT

ABSTRACT Hereditary hyperferritinemia-cataract syndrome is an autosomal dominant genetic disorder associated with mutations in the 5'UTR region of the ferritin light chain gene. These mutations cause the ferritin levels to increase even in the absence of iron overload. Patients also develop bilateral cataract early due to accumulation of ferritin in the lens, and many are misdiagnosed as having hemochromatosis and thus not properly treated. The first cases were described in 1995 and several mutations have already been identified. However, this syndrome is still a poorly understood. We report two cases of unrelated Brazilian families with clinical suspicion of the syndrome, which were treated in our department. For the definitive diagnosis, the affected patients, their parents and siblings were submitted to Sanger sequencing of the 5'UTR region for detection of the ferritin light gene mutation. Single nucleotide polymorphism-like mutations were found in the affected patients, previously described. The test assisted in making the accurate diagnosis of the disease, and its description is important so that the test can be incorporated into clinical practice.


RESUMO A síndrome hereditária hiperferritinemia-catarata é uma doença genética autossômica dominante associada a mutações na região 5'UTR do gene da cadeia leve da ferritina. Estas mutações elevam os níveis de ferritina, mesmo na ausência de sobrecarga de ferro. Os pacientes também desenvolvem catarata bilateral precocemente, devido ao acúmulo de ferritina no cristalino, e muitos são erroneamente diagnosticados como portadores de hemocromatose, sendo tratados de maneira inadequada. Os primeiros casos foram descritos em 1995, e diversas mutações já foram identificadas. Entretanto, essa síndrome ainda é pouco conhecida. Relatamos dois casos de famílias brasileiras, não relacionadas, com suspeita clínica da síndrome, que foram atendidas em nosso serviço. Para o diagnóstico definitivo, os pacientes afetados, seus pais e irmãos foram submetidos à pesquisa de mutação do gene ferritina, por sequenciamento de Sanger da região 5'UTR. Foram encontradas mutações do tipo polimorfismo de nucleotídeo único nos pacientes afetados, já descritas anteriormente. O teste auxiliou no diagnóstico preciso da doença e é importante ser divulgado, para ser incorporado na prática clínica.


Subject(s)
Humans , Male , Child, Preschool , Child , Apoferritins/blood , Cataract/congenital , Iron Metabolism Disorders/congenital , Iron/blood , Syndrome , Cataract/genetics , Cataract/blood , Brazil , Iron Metabolism Disorders/genetics , Iron Metabolism Disorders/blood , Mutation/genetics
11.
Einstein (Säo Paulo) ; 15(4): 465-469, Oct.-Dec. 2017. tab
Article in English | LILACS | ID: biblio-891424

ABSTRACT

ABSTRACT Objective: To analyze the quality and quantity of data in the questionnaires and in request forms for magnetic resonance imaging. Methods: This retrospective study was conducted with data from 300 medical records. The research used the following data from the questionnaires: patient age, reason for the magnetic resonance imaging, reason for placing the breast implant, report of any signs or symptoms, time elapsed since surgery to place the current breast implant, replacement implant surgery, chemotherapy, and/or radiation therapy treatments. From the magnetic resonance imaging request forms, information about the breast implant, the implant placement surgery, patient clinical information and ordering physician specialty were verified. Results: The mean age of patients was 48.8 years, and the mean time elapsed since breast implant surgery was 5 years. A total of 60% of women in the sample were submitted to aesthetic surgery, while 23.7% were submitted to chemotherapy and/or radiation therapy. In the request forms, 23.7% of physicians added some piece of information about the patient, whereas 2.3% of them informed the type of implant and 5.2% informed about the surgery. Conclusion: The amount of information in the magnetic resonance imaging request forms is very limited, and this may hinder quality of radiological reports. Institutional and technological measures should be implemented to encourage the requesting physicians and radiologists to share information.


RESUMO Objetivo: Analisar a qualidade e a quantidade de dados que constam nos questionários e nas requisições médicas de exame de ressonância magnética. Métodos: Estudo retrospectivo com 300 prontuários de pacientes. Dos questionários, foram utilizados os seguintes dados: idade, razão para a realização do exame, motivo para a colocação da prótese, referência a sinal ou sintoma, tempo decorrido desde a cirurgia de implante da prótese atual, se a cirurgia foi de troca da prótese, e se foi submetida à radioterapia ou à quimioterapia. Das requisições médicas do exame, foram utilizadas informações sobre prótese mamária, cirurgia de colocação do implante, dados clínico da paciente e especialidade do médico requisitante. Resultados: A média da idade das pacientes foi de 48,8 anos, e o tempo decorrido desde a colocação do implante foi de 5 anos, na média. Foram submetidas à cirurgia estética 60% das mulheres da amostra, e 23,7% tinham sido submetidas à quimioterapia e/ou radioterapia. Na requisição médica, 23,7% dos médicos inseriram algum dado, sendo que 2,3% informaram o tipo de implante e 5,2% sobre a cirurgia realizada. Conclusão: A quantidade de informações contidas nas requisições médicas foi baixa, o que pode comprometer a qualidade do laudo radiológico. Medidas institucionais e tecnológicas deveriam ser adotadas para estimular o intercâmbio de informações entre o médico solicitante e o médico radiologista.


Subject(s)
Humans , Female , Adolescent , Adult , Aged , Aged, 80 and over , Young Adult , Referral and Consultation/statistics & numerical data , Magnetic Resonance Imaging/statistics & numerical data , Surveys and Questionnaires , Breast Implants/adverse effects , Silicones/adverse effects , Time Factors , Medical Records/standards , Retrospective Studies , Medical Order Entry Systems , Middle Aged
12.
Einstein (Säo Paulo) ; 15(3): 355-358, July-Sept. 2017.
Article in English | LILACS | ID: biblio-891404

ABSTRACT

ABSTRACT Acute myeloid leukemia is a hematopoietic stem cell neoplastic disease associated with high morbidity and mortality. The presence of FLT3 internal tandem duplication mutations leads to high rates of relapse and decreased overall survival. Patients with FLT3 internal tandem duplication are normally treated with hematopoietic stem cell transplantation in first complete remission. Nevertheless, the incidence of post-transplant relapse is considerable in this group of patients, and the management of this clinical condition is challenging. The report describes the outcomes of patients with FLT3 internal tandem duplication positive acute myeloid leukemia who relapsed after allogeneic hematopoietic stem cell transplantation and were treated with the combination of re-induction chemotherapy, donor lymphocyte infusion, sorafenib and azacitidine. Three cases are described and all patients achieved prolonged complete remission with the combined therapy. The combination of induction chemotherapy followed by donor lymphocyte infusion, and the maintenance with azacitidine and sorafenib can be effective approaches in the treatment of post-hematopoietic stem cell transplant and relapsed FLT3 internal tandem duplication positive acute myeloid leukemia patients. This strategy should be further explored in the context of clinical trials.


RESUMO A leucemia mieloide aguda é uma doença neoplásica de células-tronco hematopoiéticas com alta morbimortalidade. A presença de mutações de duplicação em tandem de FLT3 leva a altas taxas de recorrência e a menor sobrevida global. Os pacientes com duplicação em tandem de FLT3 são normalmente tratados com transplante de células-tronco hematopoiéticas na primeira remissão completa. No entanto, a incidência de recidiva pós-transplante é considerável neste grupo de pacientes, e a conduta, nestes casos, é um desafio. O relato descreve os resultados do tratamento de pacientes com leucemia mieloide aguda positiva e duplicação em tandem de FLT3 que recidivaram depois do transplante alogênico de células-tronco hematopoiéticas e que foram tratados com combinação de quimioterapia de reindução, infusão de linfócitos de doador, sorafenib e azacitidina. São descritos três casos, e todos os pacientes apresentaram remissão completa prolongada com a terapia combinada. A combinação de quimioterapia de indução, seguida de infusão de linfócitos do doador, e a manutenção com azacitidina e sorafenib podem ser abordagens eficazes no tratamento da recorrência pós-transplante em pacientes com leucemia mieloide aguda e duplicação em tandem de FLT3. Essa estratégia deve ser mais explorada no contexto de ensaios clínicos.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Phenylurea Compounds/administration & dosage , Azacitidine/administration & dosage , Leukemia, Myeloid, Acute/therapy , Niacinamide/analogs & derivatives , Lymphocyte Transfusion , fms-Like Tyrosine Kinase 3/genetics , Induction Chemotherapy , Antineoplastic Agents/administration & dosage , Recurrence , Leukemia, Myeloid, Acute/genetics , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Treatment Outcome , Niacinamide/administration & dosage , Combined Modality Therapy/methods , Neoplasm Recurrence, Local/therapy
14.
Rev. bras. hematol. hemoter ; 38(4): 277-278, Oct.-Dec. 2016. graf
Article in English | LILACS | ID: biblio-829945

Subject(s)
Biography , Hematology
15.
Rev. Assoc. Med. Bras. (1992) ; 62(supl.1): 29-33, Oct. 2016. tab, graf
Article in English | LILACS | ID: biblio-829561

ABSTRACT

SUMMARY Objective: To review and discuss the literature on hematopoietic stem cell transplantation (HSCT) with haploidentical donors in Brazil. Method: Literature review. Results: The haploidentical hematopoietic stem cell transplantations have become a safe option in hematology since the 80s, with the possibility of ex-vivo T-cell depletion. However, its broad use worldwide occurred with the advent of haploidentical nonmyeloablative transplants using in vivo T-cell depletion with the administration of post-transplant cyclophosphamide. The results were encouraging, despite the increased risk of infection and post-transplantation recurrence. Recent publications on acute myeloid leukemia, myelodysplastic syndrome and Hodgkin’s lymphoma have shown similar results among haploidentical, unrelated and related full-match transplants. Obviously, these findings of retrospective studies should be confirmed by clinical trials. Conclusions: Transplantation with haploidentical donor has shown to be feasible in Brazil and the first publications and results are showing encouraging results.


RESUMO Objetivo: Revisar e discutir a literatura sobre transplantes de células-tronco hematopoiéticas (TCTH) com doador haploidêntico no Brasil. Métodos: Revisão da literatura médica. Resultados: Os transplantes haploidênticos de células-tronco hematopoiéticas tornaram-se uma opção segura na hematologia a partir dos anos 1980, com a possibilidade de depleção de células T ex-vivo. No entanto, sua ampla utilização em todo mundo ocorreu após os trabalhos com os transplantes haploidênticos não mieloablativos, com depleção de células T in-vivo, utilizando ciclofosfamida pós-transplante. Os resultados se mostraram encorajadores, apesar do maior risco de infecções e recidiva pós-transplante. Estudos em determinadas patologias, principalmente na leucemia mieloide aguda, mielodisplasia e linfoma de Hodgkin, mostram resultados semelhantes entre transplantes haploidênticos e não aparentados e aparentados totalmente compatíveis. Logicamente, esses achados de estudos retrospectivos precisam ser confirmados por estudos clínicos. Conclusões: No Brasil, a modalidade de transplante com doador haploidêntico se mostrou factível e as primeiras publicações e resultados mostram resultados animadores.


Subject(s)
Humans , Tissue Donors , Hematopoietic Stem Cell Transplantation/methods , Donor Selection/methods , Histocompatibility , Time Factors , Brazil , Treatment Outcome , Risk Assessment , Hematopoietic Stem Cell Transplantation/mortality
16.
Rev. bras. hematol. hemoter ; 38(1): 1-6, Jan.-Feb. 2016. tab, graf
Article in English | LILACS | ID: lil-777433

ABSTRACT

ABSTRACT Background: Successful transfusion of platelet refractory patients is a challenge. Many potential donors are needed to sustain human leukocyte antigen matched-platelet transfusion programs because of the different types of antigens and the constant needs of these patients. For a highly mixed population such as the Brazilian population, the pool size required to provide adequate platelet support is unknown. Methods: A mathematical model was created to estimate the appropriate size of an unrelated donor pool to provide human leukocyte antigen-compatible platelet support for a Brazilian population. A group of 154 hematologic human leukocyte antigen-typed patients was used as the potential patient population and a database of 65,500 human leukocyte antigen-typed bone marrow registered donors was used as the donor population. Platelet compatibility was based on the grading system of Duquesnoy. Results: Using the mathematical model, a pool containing 31,940, 1710 and 321 donors would be necessary to match more than 80% of the patients with at least five completely compatible (no cross-reactive group), partial compatible (one cross-reactive group) or less compatible (two cross-reactive group) donors, respectively. Conclusion: The phenotypic diversity of the Brazilian population has probably made it more difficulty to find completely compatible donors. However, this heterogeneity seems to have facilitated finding donors when cross-reactive groups are accepted as proposed by the grading system of Duquesnoy. The results of this study may help to establish unrelated human leukocyte antigen-compatible platelet transfusions, a procedure not routinely performed in most Brazilian transfusion services.


Subject(s)
Humans , Bone Marrow , HLA Antigens , Platelet Transfusion
18.
Säo Paulo med. j ; 133(6): 471-479, Nov.-Dec. 2015. tab
Article in English | LILACS | ID: lil-770158

ABSTRACT

CONTEXT AND OBJECTIVES: Chronic myeloid leukemia (CML) requires strict daily compliance with oral medication and regular blood and bone marrow control tests. The objective was to evaluate CML patients' perceptions about the disease, their access to information regarding the diagnosis, monitoring and treatment, adverse effects and associations of these variables with patients' demographics, region and healthcare access. DESIGN AND SETTING: Prospective cross-sectional study among CML patients registered with the Brazilian Lymphoma and Leukemia Association (ABRALE). METHODS: CML patients receiving treatment through the public healthcare system were interviewed by telephone. RESULTS: Among 1,102 patients interviewed, the symptoms most frequently leading them to seek medical care were weakness or fatigue. One third were diagnosed by means of routine tests. The time that elapsed between first symptoms and seeking medical care was 42.28 ± 154.21 days. Most patients had been tested at least once for Philadelphia chromosome, but 43.2% did not know the results. 64.8% had had polymerase chain reaction testing for the BCR/ABL gene every three months. 47% believed that CML could be controlled, but 33.1% believed that there was no treatment. About 24% reported occasionally stopping their medication. Imatinib was associated with nausea, cramps and muscle pain. Self-reported treatment adherence was significantly associated with normalized blood count, and positively associated with imatinib. CONCLUSIONS: There is a lack of information or understanding about disease monitoring tools among Brazilian CML patients; they are diagnosed quickly and have good access to treatment. Correct comprehension of CML control tools is impaired in Brazilian patients.


CONTEXTO E OBJETIVOS: Leucemia mieloide crônica (CML) exige estrita adesão à medicação oral e ao monitoramento do sangue e da medula. O objetivo foi avaliar percepções de pacientes com leucemia mieloide crônica (LMC) sobre a doença, seu acesso à informação sobre diagnóstico, monitoramento e tratamento, efeitos adversos e a associação destes com dados demográficos, geográficos e de acesso a tratamento. DESENHO E LOCAL: Estudo prospectivo transversal realizado com pacientes de LMC cadastrados na Associação Brasileira de Leucemia e Linfoma (Abrale). MÉTODOS: Pacientes com LMC recebendo tratamento do sistema público de saúde foram entrevistados por telefone. RESULTADOS: Entre os 1.102 pacientes entrevistados, os sintomas mais frequentemente levando à busca de consulta foram fraqueza e fadiga. Um terço foi diagnosticado por exames de rotina. O tempo entre sintoma inicial e procura por ajuda foi de 42,28 ± 154,21 dias. A maioria foi testada pelo menos uma vez para o cromossomo Filadélfia, mas 43,2% não sabiam os resultados. 64,8% fizeram exame de reação em cadeia da polimerase para o gene BCR/ABL a cada três meses. 47% acreditavam que LMC pode ser controlada, mas 33,1% acham que não há tratamento. Cerca de 24% disseram que ocasionalmente interrompem o tratamento. Imatinibe associou-se com náusea, câimbra e dor muscular. Aderência auto-reportada associou-se significativamente com hemograma normal e positivamente com uso de imatinibe. CONCLUSÕES: Falta informação ou compreensão sobre monitoramento entre pacientes com LMC; eles recebem diagnóstico rapidamente e têm bom acesso ao tratamento. A correta compreensão das ferramentas de controle em LMC está prejudicada entre eles.


Subject(s)
Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Young Adult , Health Knowledge, Attitudes, Practice , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/diagnosis , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/therapy , Antineoplastic Agents/therapeutic use , Brazil , Cross-Sectional Studies , Health Services Accessibility , Imatinib Mesylate/therapeutic use , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/genetics , Medication Adherence , Perception , Philadelphia Chromosome , Prospective Studies , Socioeconomic Factors , Statistics, Nonparametric , Time Factors
19.
Rev. bras. hematol. hemoter ; 37(1): 3-4, Jan-Feb/2015.
Article in English | LILACS | ID: lil-741868

Subject(s)
Leukemia, Myeloid
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