Scielo RSS <![CDATA[Revista Brasileira de Hematologia e Hemoterapia]]> http://www.scielo.br/rss.php?pid=1516-848420030001&lang=en vol. 25 num. 1 lang. en <![CDATA[SciELO Logo]]> http://www.scielo.br/img/en/fbpelogp.gif http://www.scielo.br <![CDATA[<B>The treatment of Acute Myeloid Leukemia in Brazil</B>: <B>how far have we already advanced and what can we improve?</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100001&lng=en&nrm=iso&tlng=en <![CDATA[<B>Quality of life in patients submitted to allogeneic bone marrow transplantation</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100002&lng=en&nrm=iso&tlng=en <![CDATA[<B>A functional assessment of adolescents who were recipients of bone marrow transplantation</B>: <B>a prospective study</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100003&lng=en&nrm=iso&tlng=en The aim of this study was to assess the physical performance (Karnofsky or Lansky's Performance Scale), as well as the social/familial, emotional, functional and cancer treatment by bone marrow transplant (FACT-BMT battery). Subjects were all adolescents (13 to 20 years of age) who underwent a transplant at the Bone Marrow Transplant Unit (Serviço de Transplante de Medula Óssea) of the University Hospital (Hospital de Clínicas) of Universidade Federal do Paraná, (STMO-HC-UFPR) in the calendar year (January to December) of 1999. The initial sample consisted of thirteen patients (seven male, six female), of whom four had a previous diagnosis of a neoplastic condition and nine of a non-neoplastic condition. Allografts were used in all cases. The study instruments and scales were applied twice: firstly, in the month prior to the procedure and secondly, on the hundredth day afterwards. Three patients died before the second assessment was carried out. Therefore, only 10 patients completed the study and were the subject of the full analysis. Results show that although 4/10 of the patients had an improved physical performance as shown by the Karnofsky and Lansky's Performance Scale, 9/10 reported difficulties to resume day-to-day activities and chores (professional, educational and homework), as shown by the FACT-BMT battery. In other domains assessed, results showed variations between the pre and post procedures but without reaching statistical significance. It could be concluded that the therapeutic intervention in itself may affect the quality of life of the subjects. It has to be said, however, that this study was carried out within the frame of continuous medical treatment and this may have acted as a confounder. Further long-term longitudinal studies are, therefore, required to improve our understanding of these issues.<hr/>O estudo teve por objetivo analisar o desempenho físico (Escala de Desempenho de Karnofsky ou de Lansky), assim como o desempenho sociofamiliar, emocional, funcional, relacionamento médico/paciente e preocupações adicionais (Escala de Avaliação Funcional para Terapia de Câncer - Transplante de Medula Óssea - FACT-BMT) de todos os adolescentes (13 a 20 anos) submetidos a transplante no Serviço de Transplante de Medula Óssea do Hospital de Clínicas da Universidade Federal do Paraná, (STMOHC- UFPR) no período de janeiro a dezembro de 1999. Inicialmente eram 13 pacientes (sete homens, seis mulheres); quatro com diagnóstico prévio de patologia neoplásica e nove não-neoplásica, levando em conta que todos foram submetidos a transplante alogênico. Os instrumentos de coleta de dados foram aplicados em dois momentos: durante o mês que antecedeu o transplante e no centésimo dia após o mesmo. Três pacientes do grupo inicial foram a óbito antes da segunda coleta. Assim, apenas dez completaram as duas etapas da avaliação e constituíram a amostra de análise (n= 10). A avaliação dos resultados demonstrou que 4/10 dos pacientes apresentaram melhoras em relação ao desempenho físico (resultados da Escala de Desempenho de Karnofsky e de Lansky), no entanto 9/10 relataram dificuldades para reassumir as atividades regulares (profissional, educacional e domésticas), resultados da FACT-BMT. Os demais tópicos avaliados demonstraram oscilações do pré para o pós-TMO, porém de baixa amplitude e sem diferença estatística significativa. Pode-se concluir que a intervenção em si compromete parcialmente a qualidade de vida dos pacientes. No entanto, é preciso considerar o fato da avaliação ter sido realizada no período em que os pacientes ainda estavam sob tratamento médico contínuo, uma vez que esta contextualização é uma variável interferente. Assim, estudos futuros devem ocorrer para avaliar a evolução longitudinal desse processo. <![CDATA[<b>Acute Myelogenous Leukemia</b>: <b>two decades overview - </b><b>Hematology</b><b> </b><b>Service</b><b> </b><b>Hospital</b><b> de Clínicas de </b><b>Porto Alegre</b><b> - RS</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100004&lng=en&nrm=iso&tlng=en Nos últimos vinte anos, nosso serviço recebeu os pacientes com a suspeita de Leucemia Mielóide Aguda (LMA) provenientes de todas as regiões do estado. Entre março de 1980 e dezembro de 1999 analisamos 195 pacientes com idades superior a 12 e inferior a 70 anos, apresentando LMA "de novo" excetuando o subtipo M3. Na década de 80 foram registrados 102 pacientes: 47 homens e 55 mulheres. Destes, 84 receberam quimioterapia de indução com Citarabina e Daunorrubicina (esquema "7+3"), resultando no índice de remissão de 51% (43/84). As médias de sobrevidas livre de doença e global foram dez meses em 35% e 12 meses em 13% respectivamente. De jan/90 a dez/93 houve 41 novos diagnósticos e todos foram submetidos à quimioterapia com esquema "7+3" atingindo a taxa de 66% de remissão. As sobrevidas livre de doença e global foram estatisticamente (p<0.001) superiores com 17,6 meses em 41% e 22 meses em 17,5% dos pacientes. Em 1994 foi instituído novo protocolo orientado pelo grupo cooperativo brasileiro de estudos de leucemia (CBEL), preconizando a indução com Citarabina +Idarrubicina, seguido dos ciclos de consolidação e dois ciclos de intensificação com altas doses. Entre 1994-99 foram incluídos 52 pacientes neste protocolo, cujo índice de remissão foi 73% (p=0,002). Em 50% destes pacientes, a sobrevida livre de doença alcançou 23,3 meses, enquanto a sobrevida global foi de 26 meses em 25%. As sobrevidas livre de doença e global, neste último período, tiveram diferenças estatisticamente significativas comparadas aos anos anteriores. Observamos uma melhora nas taxas de remissão e de sobrevida na última década, mesmo assim a sobrevida longa livre de eventos permanece inferior a 30%. Nossos resultados são equivalentes aos demais centros nacionais que tratam LMA e corroboram os dados da literatura.<hr/>We have treated many AML patients in the last 20 years. We followed, between March 1980 and December 1999, 195 patients with ages ranging from 12 to 70 years and presenting "de novo" AML, excluding the M3 subtype. In the eighties, 102 patients are on record: 47 males and 55 females. Among these, 84 received induction chemotherapy with Cytarabin plus Daunorrubicin (7+3), resulting in a 51% (43/84) remission rate. The average disease-free and overall survival was 35% at 10 months and 13% at 12 months. Forty-one new diagnoses were performed from January 1990 and December 1993, all patients were submitted to the "7+3" chemotherapy protocol and a 66% remission rate was obtained. The disease free and overall survival rates were statistically (p < 0.001) higher with 41% of the patients at 7.6 months and 17.5% at 22 months. In 1994 a new protocol was introduced under the guidance of the Brazilian Cooperative Group on Leukemia Studies (GCBEL). It recommended induction with Cytarabin + Idarrubicin followed by consolidation and two intensification cycles with high doses of Cytarabin. Between 1994 and 1999, 52 patients were included in this new protocol and the remission rate was 73% (p = 0.002). In 50% of these patients disease free survival was about 23.3 months, while the overall survival was 25% at 26 months. Both disease free survival and overall survival rates in the last period showed statistically significant differences when compared to the previous years. We observed a progressive improvement in remission and survival rates in the last decades, however the prolonged event-free survival stayed below 30%. Our results are similar to the national centers that treat AML and in accordance with the literature data. <![CDATA[<B>Assessment of phagocytic function in remnants of subtotal spleen implantation</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100005&lng=en&nrm=iso&tlng=en Para diminuir os efeitos adversos da esplenectomia total, abordagens conservadoras devem ser tentadas, quando for possível. Esplenectomia subtotal e auto-implantes esplênicos são boas alternativas nessa situação. O objetivo do presente estudo foi comparar as funções fagocitárias do pólo superior remanescente de esplenectomia subtotal e de tecido esplênico implantado no omento maior. Esta investigação foi conduzida em 35 ratos Holtzman adultos de ambos os sexos. Todos os animais foram submetidos a esplenectomia subtotal preservando o pólo superior suprido pelos vasos esplenogástricos e a auto-implante do segmento esplênico removido, no omento maior. A função fagocitária foi verificada por dois métodos diferentes: cintilografia com enxofre coloidal marcado com 99mTc e contagem, ao microscópio, de macrófagos que continham carbono coloidal. Durante os três primeiros meses, a fagocitose foi maior no pólo superior remanescente. Após esse período, não houve diferença entre o pólo superior e o auto-implante. Concluindo, o remanescente de esplenectomia subtotal e o auto-implante esplênico mantêm a função de filtração do baço mediante remoção de partículas coloidais do sangue.<hr/>In order to diminish the adverse effects of total splenectomy, a conservative approach must be performed whenever possible. Subtotal splenectomy and splenic autotransplantation are good alternatives in such cases. The purpose of the present study was to compare the phagocytic function of the upper pole remnant of subtotal splenectomy and of autologous spleen tissue implanted into the greater omentum. This investigation was carried out on 35 adult Holtzman rats of both sexes. All animals were submitted to subtotal splenectomy, preserving the upper pole supplied by the splenogastric vessels and autotransplantation of the removed splenic segment on the greater omentum. The phagocytic function of the splenic remnants was verified by two different methods: scintigraphy with 99mTc sulfur colloid and microscopic counting of macrophages containing carbon colloid. During the first three months, the phagocytosis was higher in the remnant upper pole. After this time, there was no difference between the upper pole and the splenic autotransplant. In conclusion, the remnant of subtotal splenectomy and the splenic autotransplant maintain the filtering function of the spleen by removing colloid particles from the blood. <![CDATA[<B>Seroprevalence and immunophenotypic profile of T lymphocyte cells in human T lymphotropic virus seropositive individuals</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100006&lng=en&nrm=iso&tlng=en O vírus linfotrópico de células T humana (HTLV) é transmitido por transfusões, uso compartilhado de agulhas contaminadas, aleitamento e contato sexual. A prevalência varia de acordo com a região geográfica, grupo racial e população estudada. Cerca de 1% a 4% dos indivíduos infectados desenvolvem algum tipo de doença em decorrência da infecção. É reconhecida a associação entre o HTLV-I e leucemia de células T do adulto e paraparesia espástica tropical (PET). Embora a maioria dos portadores permaneça assintomática, existem evidências de comprometimento funcional da resposta imune celular. Os objetivos desse trabalho foram avaliar a prevalência de soropositividade para HTLV-I/II na população de doadores de sangue do HEMOCE e analisar o perfil imunofenotípico de células linfóides circulantes em 26 doadores soronegativos, 11 soropositivos para HTLV-I sintomáticos e 24 assintomáticos, comparando-os entre si. A prevalência da soropositividade para HTLV-I/II foi de 0,66%. No grupo de indivíduos contaminados pelo HTLV-I houve predomínio do sexo feminino e a maior média de idade. O grupo soropositivo apresentou menor valor de hemoglobina e o grupo sintomático evidenciou contagem de neutrófilos significativamente mais elevada. A contagem média de linfócitos não diferiu entre os grupos. A análise imunofenotípica mostrou que os valores médios de células CD3+, CD4+, CD8+ e relação CD4/CD8 não diferiram significativamente entre os grupos. Uma elevação de células CD8+ no grupo soropositivo foi observada embora não alcançasse significância estatística. A ativação de linfócitos CD8+ está envolvida na patogênese das doenças associadas ao HTLV-I. A definição do valor preditivo desse achado requer confirmação posterior.<hr/>Human T lymphotropic virus (HTLV) can be transmitted by transfusions of cellular blood products, shared use of contaminated syringes, breast feeding and sexual intercourse. The prevalence of the infection varies according to geographic region, racial group, and population under risk. About 1% to 4% of the infected individuals develop some form of infection-related disease. The association of HTLV-I with Adult T-Cell Leukaemia, as well as the Tropical Spastic Paraparesis, is presently well recognised. Although most HTLV-I-infected individuals remain asymptomatic, there are indications that cellular immune responses are functionally impaired. The aims of this study were to determine the prevalence of HTLV-I/II seropositivity among blood donors in HEMOCE and analyse the immunophenotypic profile of peripheral lymphocytes in 26 HTLV-I seronegative blood donors, 11 symptomatic and 24 asymptomatic HTLV-I soropositive individuals. The prevalence of HTLV-I/II was 0,66%. In the infected group a predominance of females was observed as well as a higher average age. The mean hemoglobin value was found to be significantly lower in this group and the mean polymorphonuclear neutrophil count was significantly higher among the symptomatic individuals. The mean lymphocyte and platelet count were not significantly different between the groups. Immunophenotyping evaluation revealed that the mean CD3+, CD4+, CD8+ T cell counts, as well as the CD4+/CD8+ ratio were not significantly different between the groups. A slightly higher mean CD8+ T lymphocyte count was observed in the seropositive individuals, although it did not reach statistical significance. The activation of CD8+ subset is known to be part of pathogenesis of HTLV-I-related diseases. The predictive value of this immunological finding needs further and long-range studies. <![CDATA[<B>Flow cytometric characterization of platelet activation in platelet concentrates</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100007&lng=en&nrm=iso&tlng=en A caracterização da expressão das glicoproteínas de superfície plaquetária e superfície pró-coagulante por citometria de fluxo é uma técnica sensível para identificação da ativação plaquetária em concentrados de plaquetas. A ativação plaquetária pode desencadear alterações que irão influir na viabilidade das plaquetas durante o período de estocagem. A detecção da ativação plaquetária durante o preparo e estocagem dos concentrados de plaquetas pode permitir a identificação dos mecanismos de ativação e sugerir alterações que irão influir na qualidade final do produto. A utilização isolada de anticorpos monoclonais específicos para plaquetas ativadas pode levar a erros de interpretação. Assim, é aconselhável o uso de painéis de anticorpos capazes de avaliar a migração das glicoproteínas IIb-IIIa e Ib-IX, o processo de degranulação e a formação de superfície pró-coagulante.<hr/>Flow cytometric characterization of platelet membrane glycoproteins expression and procoagulant surface is a sensitive technique to identify platelet activation in platelet concentrates. Platelet activation could contribute to platelet storage lesion reducing platelet viability and its hemostatic function. Detection of activation in platelet concentrates may help to determine the mechanism by which platelets activate during preparation and storage. It may also suggest interventions to prevent and reduce the platelet storage lesion. A monoclonal antibody panel that evaluate glycoproteins IIb-IIIa and Ib-IX migration, platelet degranulation and platelet procoagulant surface would provide more information about platelet activation than activation-dependent monoclonal antibodies alone. <![CDATA[<B>Molecular aspects of ABO Blood Group System</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100008&lng=en&nrm=iso&tlng=en O sistema ABO é o mais importante grupo sangüíneo na medicina transfusional. O gene ABO codifica as glicosiltransferases responsáveis pela transferência dos resíduos específicos de açúcar, GalNaca1-3 e Gala 1-3, ao substrato H e os convertem ao antígeno A ou B respectivamente. A estrutura do DNA dos três principais alelos do sistema ABO, A¹, B e O foi primeiramente descrita em 1990. Os avanços da genética molecular permitiram o entendimento da base molecular dos genes ABO e o conhecimento do polimorfismo dos alelos comuns a esse locus. Essa revisão tem como objetivo o estudo dos alelos variantes desse sistema, assim como a compreensão das mutações, deleções ou rearranjo de genes responsáveis pela ocorrência de alguns dos subgrupos do sistema ABO. As técnicas mais comumente utilizadas para a genotipagem ABO também são avaliadas, bem como suas vantagens e limitações.<hr/>The ABO blood group is the most important blood group system in transfusion medicine. Antigens of the ABO system consist of A or B carbohydrate structure carried on the substrate H antigen The ABO gene is responsible for encoding for glycosyltransferases A or B that defines which specific carbohydrate is added to the end of H substance oligosaccharide chains, GalNacalpha1-3 and Galalpha1-3, respectively. The DNA structure of the three major alleles of the human blood group ABO system, A¹ and B, was first described in 1990. Advances of molecular genetics have allowed understanding of the molecular basis of the ABO blood group system and the knowledge of the common alleles polymorphisms of this locus. This review article has the purpose of describing the variants of these alleles and the underlying mutations, deletions or rearrangement of the genes responsible for the occurrence of ABO subgroups and O transferases inactivation. Finally, various methods available for ABO genotyping are also evaluated, as well as its advantages and limitations. <![CDATA[<B>Oral Iron chelation therapy in Beta-Thalassaemia</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100009&lng=en&nrm=iso&tlng=en A quelação do ferro com deferroxamina (DFO) melhorou dramaticamente o prognóstico de pacientes com beta talassemia. A DFO reduz os estoques de ferro, bem como a morbidade e mortalidade destes pacientes. No entanto, a necessidade de infusões parenterais prolongadas estimulou a busca por novos quelantes. Deferiprone (DP) é o único quelante oral em uso clínico e já avaliado em estudos clínicos. A terapia combinada com DP e DFO surgiu como alternativa para pacientes talassêmicos. Ela tem o potencial de minimizar os efeitos adversos, aumentar a adesão e a eficácia, e, talvez, atingir compartimentos distintos de ferro no organismo. A disponibilização de novos quelantes e métodos de avaliação do ferro no organismo poderá permitir a individualização do tratamento quelante.<hr/>Iron chelation therapy with desferrioxamine (DFO) has dramatically improved the outlook in beta-thalassaemia patients. DFO reduces tissue iron stores, prevents iron-induced organ damage, and reduces morbidity and mortality. However, the need for prolonged subcutaneous portable pump infusions, high cost, and patient noncompliance have prompted the development of new iron chelators. Deferiprone, is the only oral iron chelator in clinical use and has been studied in large long-term clinical trials. Combination therapy with deferiprone and DFO has emerged as a new alternative for beta thalassaemia patients. Combination therapy may minimize side effects, improve compliance, enhance iron excretion and target specific iron pools. The availability of new chelating compounds and new methods for body-iron store evaluation may lead to individualized therapy in the future. <![CDATA[<b>Amnaphtone in the treatment of nosebleed</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100010&lng=en&nrm=iso&tlng=en Nasal haemorrhage or nosebleed, is the most common of haemorrhages due to the intense vasculization of the nose, the fragility of the nasal mucous membrane and its vulnerability to traumas and irritant agents. The aim of this study is to report on a reduction of episodes of nosebleed in two patients with the use of amnophtone. A 34-year-old male patient and a 44- year-old female patient presented with histories of two or more nosebleeds per week, despite treatment during the acute phase. They both sought medical assistance for other vascular complaints, which were suggestive of vascular insufficiency as, during the physical examination petechia lesions in the lower limbs were detected. They were treated with 75 mg of amnaphtone three times daily. One week after, at the follow-up, they reported an improvement of the vascular symptoms, however, more markedly was the significant improvement in relation to the nosebleeds which were frequent but had not been reported in the first consultation. In conclusion, amnaphtone demonstrated to be efficient in the prevention of recurrence of nosebleed in these patients. <![CDATA[<b>Unsuccessful treatment of sideroblastic anaemia with chloroquine</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100011&lng=en&nrm=iso&tlng=en As anemias sideroblásticas representam um grupo de moléstias heterogêneas. O seu tratamento é restrito e baseado em transfusões de sangue. Relatos descrevem um potencial benefício no uso da cloroquina na anemia sideroblástica (AS). Neste estudo, seis pacientes com AS foram tratados pelo período de seis meses com 300g/dia de cloroquina. Epigastralgia foi um sintoma freqüente nos pacientes do estudo obrigando à interrupção do tratamento em dois deles. A necessidade transfusional permaneceu a mesma em dois pacientes e não ocorreu em nenhum paciente o aumento dos níveis de hemoglobina ou a redução do percentual de sideroblastos em anel. A ausência de resultados e os efeitos colaterais sugerem que o uso de cloroquina seja inadequado para o tratamento de AS. <![CDATA[<B>Evaluation of the blood groups and hemoglobin phenotypes in a group of university students of São José do Rio Preto, SP, Brazil</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100012&lng=en&nrm=iso&tlng=en The studies of the phenotypes of the blood groups are important for blood transfusions, for obstetrics, neonatology and law medicine, apart from its application in anthropology where it can be used as genetic markers in population studies. Works with hemoglobin polymor-phisms as a genetic marker of populations has been increased over the last few years, particularly for different population groups of Brazil. In the investigation the hemoglobin polymorphisms of 200 university students from São José do Rio Preto, Brazil were studied to establish a possible relation between the different phenotypes. Statistical analysis of the phenotypes showed that the differences observed were not significant, without a relationship among the polymorphisms of blood groups and hemoglobins. <![CDATA[<B>Prognostic tests of rejection and graft-versus-host disease in allogeneic hematopoietic stem cell transplantation with HLA-identical donor</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100013&lng=en&nrm=iso&tlng=en The studies of the phenotypes of the blood groups are important for blood transfusions, for obstetrics, neonatology and law medicine, apart from its application in anthropology where it can be used as genetic markers in population studies. Works with hemoglobin polymor-phisms as a genetic marker of populations has been increased over the last few years, particularly for different population groups of Brazil. In the investigation the hemoglobin polymorphisms of 200 university students from São José do Rio Preto, Brazil were studied to establish a possible relation between the different phenotypes. Statistical analysis of the phenotypes showed that the differences observed were not significant, without a relationship among the polymorphisms of blood groups and hemoglobins. <![CDATA[<B>Allogeneic bone marrow transplantation in patients with severe aplastic anemia younger than 12 years old, conditioned with cyclophosphamide and total body irradiation</B>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842003000100014&lng=en&nrm=iso&tlng=en The studies of the phenotypes of the blood groups are important for blood transfusions, for obstetrics, neonatology and law medicine, apart from its application in anthropology where it can be used as genetic markers in population studies. Works with hemoglobin polymor-phisms as a genetic marker of populations has been increased over the last few years, particularly for different population groups of Brazil. In the investigation the hemoglobin polymorphisms of 200 university students from São José do Rio Preto, Brazil were studied to establish a possible relation between the different phenotypes. Statistical analysis of the phenotypes showed that the differences observed were not significant, without a relationship among the polymorphisms of blood groups and hemoglobins.