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Abstract Introduction Magnetic resonance imaging (MRI) T2* technique is used to assess iron overload in the heart, liver and pancreas of thalassaemic patients. Optimal iron chelation and expected tissue iron response rates remain under investigation. The objective of this study was to analyse serum ferritin and the iron concentration in the heart, liver and pancreas measured by MRI T2*/R2* during regular chelation therapy in a real-world cohort of patients with thalassemia. Methods We evaluated thalassaemic patients ≥ 7 years old undergoing chelation/transfusion therapy by MRI and assessed serum ferritin at baseline and follow-up from 2004-2011. Results We evaluated 136 patients, 92% major thalassaemic, with a median age of 18 years, and median baseline ferritin 2.033ng/ml (range: 59-14,123). Iron overload distribution was: liver (99%), pancreas (74%) and heart (36%). After a median of 1.2 years of follow-up, the iron overload in the myocardium reduced from 2,63 Fe mg/g to 2,05 (p 0.003). The optimal R2* pancreas cut-off was 148 Hertz, achieving 78% sensitivity and 73% specificity. However, when combining the R2* pancreas cut off ≤ 50 Hertz and a ferritin ≤ 1222 ng/ml, we could reach a negative predictive value (NPV) of 98% for cardiac siderosis. Only 28% were undergoing combined chelation at baseline assessment, which increased up to 50% on follow up evaluation. Conclusions Chelation therapy significantly reduced cardiac siderosis in thalassaemic patients. In patients with moderate/severe liver iron concentration undergoing chelation therapy, ferritin levels and myocardium iron improved earlier than the liver siderosis.

https://doi.org/10.1016/j.htct.2021.01.014

Urinary retention after use of decitabine in acute myeloid leukemia: case report
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Nunes, Viviane Regina Hernandez

; Pimentel, Isabella Silva e

; Kerbauy, Mariana Nassif

; Feres, Carolina Cristina Pellegrino

; Nascimento, Jade Zezzi Martins do

; Ribeiro, Andreza Alice Feitosa

; Santos, Amanda Inacio Dias dos

; Wroclawski, Marcelo Langer

; Teixeira, Larissa Lane Cardoso

; Hamerschlak, Nelson

Hematology, Transfusion and Cell Therapy

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mar 2023, Volume 45 N. 1 Pages 110 - 112

Abstract: En | Text: En | PDF: En

https://doi.org/10.1016/j.htct.2021.04.008

Molecular profile of patients with myelofibrosis: a 10-year experience
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Dias, Lara Faria Souza

; Pereira, Carolina Leme de Moura

; Centurião, Newton de Freitas

; Nascimento, Jade Zezzi Martins do

; Ribeiro, Andreza Alice Feitosa

; Hamerschlak, Nelson

; Marques, Carolina Perrone

; Lima, Ana Carolina Vieira de

; Costa, Luana Nóbrega da

; Silva, Anderson Felipe da

; Lima, Viviane de Jesus Torres

; Kerbauy, Mariana Nassif

; Kerbauy, Lucila Nassif

; Arcuri, Leonardo Javier

; Campregher, Paulo Vidal

; Rocha, Juliana Dall´Agnol da

; Datoguia, Tarcila Santos

; Santos, Fabio Pires de Souza

einstein (São Paulo)

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2023, Volume 21 elocation eAO0100

Abstract: En | Text: En | PDF: En

ABSTRACT Objective To analyze the karyotype test and myeloid panel with next-generation sequencing findings in patients with myelofibrosis, and to compare transplant characteristics in patients referred for bone marrow transplantation. Methods Retrospective, single-center study with patients diagnosed with myelofibrosis treated at Hospital Israelita Albert Einstein between 2010 and 2020. Results A total of 104 patients with myelofibrosis were examined. Patients who had not been submitted to tests in our service were excluded. The final sample comprised 69 patients. Of these 69, 56 were submitted to karyotyping and 22 to myeloid panel with next-generation sequencing. Karyotype was normal in 60% of the patients and altered in 40%. The prevalence of high-risk molecular mutations was higher in patients referred for bone marrow transplantation (100% versus 50%). The median follow-up of transplant patients was 2.4 years and the overall survival at 2 years was 80% (95%CI: 62-100%). Conclusion The molecular analysis enables estimating the patient’s risk and thus instituting more aggressive treatment such as bone marrow transplant for patients at higher risk, being a relevant tool to guide therapy. Given the significance of molecular analysis for therapeutic decision-making in myelofibrosis, collection and disclosure of data on the prevalence of cytogenetic changes and findings of next-generation sequencing in affected patients is important.

https://doi.org/10.31744/einstein_journal/2023ao0100

GVHD treatment with extracorporeal photopheresis in Brazil: a national survey
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Fatobene, Giancarlo

; Cordeiro, Ana

; Mariano, Livia

; Silva, Marcia

; Bouzas, Luis

; Hamerschlak, Nelson

; Macedo, Maria Cristina

; Petta, Alessandra

; Funke, Vaneuza

; Novis, Yana

; Flowers, Mary E

; Rocha, Vanderson

Hematology, Transfusion and Cell Therapy

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sep 2022, Volume 44 N. 3 Pages 450 - 452

Abstract: En | Text: En | PDF: En

https://doi.org/10.1016/j.htct.2021.11.018

Progressive multifocal leukoencephalopathy in a patient with relapsed chronic lymphocytic leukemia treated with Ibrutinib
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Teixeira, Larissa Lane Cardoso

; Nunes, Viviane Regina Hernandez

; Perini, Guilherme Fleury

; Feres, Carolina Cristina Pellegrino

; Ovigli, Danielle

; Hamerschlak, Nelson

Hematology, Transfusion and Cell Therapy

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sep 2022, Volume 44 N. 3 Pages 437 - 439

Abstract: En | Text: En | PDF: En

https://doi.org/10.1016/j.htct.2020.11.006

Duffy phenotyping and FY*B-67T/C genotyping as screening test for benign constitutional neutropenia
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Barreto, Maria E.S.F

; Lipay, Mariana E

; Santos, Leandro D

; Sirianni, Marilia F. Mascarenhas

; Costa, Thiago H

; Castilho, Lilian

; Hamerschlak, Nelson

; Kutner, José M

; Bub, Carolina B

Hematology, Transfusion and Cell Therapy

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dec 2021, Volume 43 N. 4 Pages 489 - 493

Abstract: En | Text: En | PDF: En

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.

https://doi.org/10.1016/j.htct.2020.08.015

A review of hematopoietic stem cell transplantation for autoimmune diseases: multiple sclerosis, systemic sclerosis and Crohn's disease. Position paper of the Brazilian Society of Bone Marrow Transplantation
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Oliveira, Maria Carolina

; Elias, Juliana Bernardes

; Moraes, Daniela Aparecida de

; Simões, Belinda Pinto

; Rodrigues, Morgani

; Ribeiro, Andreza Alice Feitosa

; Piron-Ruiz, Lilian

; Ruiz, Milton Arthur

; Hamerschlak, Nelson

Hematology, Transfusion and Cell Therapy

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mar 2021, Volume 43 N. 1 Pages 65 - 86

Abstract: En | Text: En | PDF: En

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.

https://doi.org/10.1016/j.htct.2020.03.002 328 downloads

Investigation of MHC gamma block C4A and C4B polymorphisms in unrelated hematopoietic stem cell transplantation
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Getz, Joselito

; Goldenstein, Monica

; Bonfim, Carmem

; Funke, Vaneuza Moreira

; Colturato, Vergílio

; Hamerschlak, Nelson

; Torres, Margareth

; Sayer, David

; Boldt, Angelica

; Pasquini, Ricardo

; Pereira, Noemi Farah

Hematology, Transfusion and Cell Therapy

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sep 2020, Volume 42 N. 3 Pages 221 - 229

Abstract: En | Text: En | PDF: En

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.

https://doi.org/10.1016/j.htct.2019.06.004 460 downloads

BODY COMPOSITION IMPACT ON SURVIVAL AND TOXICITY OF TREATMENT IN PANCREATIC CANCER: CROSS-SECTIONAL PILOT STUDY
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BARRÈRE, Ana Paula Noronha

; PIOVACARI, Silvia Maria Fraga

; USÓN JUNIOR, Pedro Luiz Serrano

; GANSL, Rene Claudio

; PEREIRA, Andrea Z

; HAMERSCHLAK, Nelson

Arquivos de Gastroenterologia

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sep 2020, Volume 57 N. 3 Pages 278 - 282

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

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.

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.

https://doi.org/10.1590/s0004-2803.202000000-52 572 downloads

10.

Idiopathic thrombocytopenic purpura in a patient with situs inversus totalis: case report and literature review
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Bo, Carolina Rodrigues Dal

; Devito, Beatriz Piovesana

; Devito, Leticia Piovesana

; Papa, Gabriella Paes del

; Hamerschlak, Nelson

einstein (São Paulo)

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2020, Volume 18 elocation eRC5111

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

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.

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.

https://doi.org/10.31744/einstein_journal/2020rc5111 953 downloads

11.

Brazilian Nutritional Consensus in Hematopoietic Stem Cell Transplantation: Adults
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Barban, Juliana Bernardo

; Simões, Belinda Pinto

; Moraes, Bruna Del Guerra de Carvalho

; Anunciação, Cássia Rehem da

; Rocha, Cristiane Santos da

; Pintor, Daniela Cristina Querino

; Guerra, Daiane Cristina

; Silva, Deise Andrade

; Brandão, Edith de Castro Martins

; Kerbauy, Fábio

; Pires, Fernanda Ramos de Oliveira

; Morais, Graciele Lima

; Schmidt Filho, Jayr

; Sicchieri, Juliana Maria Faccioli

; Barroso, Karine Sampaio Nunes

; Viana, Luciana Verçoza

; Rocha, Mariana Hollanda Martins da

; Guimarães, Maysa Penteado

; Lazzari, Natalia Leonetti Couto

; Hamerschlak, Nelson

; Ramos, Patricia

; Gomes, Plinio Nascimento

; Mendonça, Priscila da Silva

; Oliveira, Raquel Candido de

; Scomparim, Renata Corrêa

; Chiattone, Ricardo

; Diez-Garcia, Rosa Wanda

; Cardenas, Thaís de Campos

; Miola, Thais Manfrinato

; Costa, Thalita Cristina de Mello

; Rocha, Vanderson

; Pereira, Andrea Z

einstein (São Paulo)

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2020, Volume 18 elocation AE4530

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

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

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.

https://doi.org/10.31744/einstein_journal/2020ae4530 3888 downloads

12.

Female genital tract chronic graft-versus-host disease: A narrative review
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Machado, Andréa Maria Novaes

; Hamerschlak, Nelson

; Rodrigues, Morgani

; Piccinato, Carla de Azevedo

; Podgaec, Sergio

; Mauad, Lenira Maria Queiroz

Hematology, Transfusion and Cell Therapy

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mar 2019, Volume 41 N. 1 Pages 69 - 75

Abstract: En | Text: En | PDF: En

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.

https://doi.org/10.1016/j.htct.2018.06.005 1658 downloads

13.

Consensus on the investigation of thrombophilia in women and clinical management
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Nascimento, Claudia Mac Donald Bley

; Machado, Andréa Maria Novaes

; Guerra, João Carlos de Campos

; Zlotnik, Eduardo

; Campêlo, Dirceu Hamilton Cordeiro

; Kauffman, Paulo

; Waksman, Hilton

; Wolosker, Nelson

; Podgaec, Sérgio

; Hamerschlak, Nelson

einstein (São Paulo)

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2019, Volume 17 N. 3 elocation eAE4510

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

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.

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.

https://doi.org/10.31744/einstein_journal/2019ae4510 10029 downloads

14.

ZAP-70 expression is associated with increased CD4 central memory T cells in chronic lymphocytic leukemia: cross-sectional study
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Correia, Rodolfo Patussi

; Silva, Flávia Amoroso Matos e

; Bacal, Nydia Strachman

; Campregher, Paulo Vidal

; Hamerschlak, Nelson

; Amarante-Mendes, Gustavo Pessini

Hematology, Transfusion and Cell Therapy

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oct 2018, Volume 40 N. 4 Pages 317 - 325

Abstract: En | Text: En | PDF: En

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.

https://doi.org/10.1016/j.htct.2018.03.008 899 downloads

15.

Ferritin light chain gene mutations in two Brazilian families with hereditary hyperferritinemia-cataract syndrome
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Petroni, Roberta Cardoso

; Rosa, Susana Elaine Alves da

; Carvalho, Flavia Pereira de

; Santana, Rúbia Anita Ferraz

; Hyppolito, Joyce Esteves

; Nascimento, Claudia Mac Donald Bley

; Hamerschlak, Nelson

; Campregher, Paulo Vidal

einstein (São Paulo)

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dec 2017, Volume 15 N. 4 Pages 492 - 495

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

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.

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.

https://doi.org/10.1590/s1679-45082017rc4006 1927 downloads

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