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Abstract To date, hydroxyurea is the only effective and safe drug that significantly reduces morbidity and mortality of individuals with Sickle cell disease. Twenty years of real-life experience has demonstrated that hydroxyurea reduces pain attacks, vaso-occlusive events, including acute chest syndrome, the number and duration of hospitalizations and the need for transfusion. The therapeutic success of hydroxyurea is directly linked to access to the drug, the dose used and adherence to treatment which, in part, is correlated to the availability of hydroxyurea. This consensus aims to reduce the number of mandatory exams needed to access the drug, prioritizing the requesting physician's report, without affecting patient safety. date disease reallife real life attacks vasoocclusive vaso occlusive events syndrome transfusion which part physicians physician s report safety

https://doi.org/10.1016/j.htct.2024.01.001

Menopause in Brazilian women with sickle cell anemia with and without hydroxyurea therapy
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Queiroz, Ana Mach

; Lobo, Clarisse Lopes de Castro

; Ballas, Samir K

Hematology, Transfusion and Cell Therapy

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Set 2021, Volume 43 Nº 3 Páginas 386 - 388

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1016/j.htct.2020.06.009 24 downloads

Association of HMIP1 C-893A polymorphism and disease severity in patients with sickle cell anemia
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Pereira-Martins, Diego A

; Domingos, Igor F

; Belini-Junior, Edis

; Coelho-Silva, Juan L

; Weinhäuser, Isabel

; Araújo, Aderson S

; Lobo, Clarisse L

; Bonini-Domingos, Claudia R

; Bezerra, Marcos A

; Lucena-Araujo, Antonio R

Hematology, Transfusion and Cell Therapy

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Set 2021, Volume 43 Nº 3 Páginas 243 - 248

Resumo: En | Texto: En | PDF: En

Abstract Introduction: Sickle cell anemia (SCA) is a Mendelian disorder with a heterogeneous clinical course. The reasons for this phenotypic diversity are not entirely established, but it is known that high fetal hemoglobin levels lead to a milder course of the disease. Additionally, genetic variants in the intergenic region HBS1L-MYB promote high levels of fetal hemoglobin into adulthood. Objective: In the present study, we investigated the HMIP1 C-839A (rs9376092) polymorphism, located at the HBS1L-MYB intergenic region block 1, in SCA patients. Method: We analyzed 299 SCA patients followed in two reference centers in Brazil. The HMIP1 C-839A (rs9376092) genotypes were determined by allele specific polymerase chain reactions. Clinical and laboratory data were obtained from patient interviews and medical records. Results: The median fetal hemoglobin levels were higher in patients with the HMIP1 C-839A (rs9376092) AA genotype (CC = 6.4%, CA = 5.6% and AA = 8.6%), but this difference did not reach significance (p = 0.194). No association between HMIP1 C-839A (rs9376092) genotypes and other clinical and laboratorial features was detected (p > 0.05). Conclusion: In summary, our data could not support the previously related association between the HMIP1 C-893A (rs9376092) polymorphism and differential fetal hemoglobin levels.

https://doi.org/10.1016/j.htct.2020.03.006 17 downloads

Consensus statement for diagnosis and treatment of paroxysmal nocturnal haemoglobinuria
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Cançado, Rodolfo D

; Araújo, Aderson da Silva

; Sandes, Alex Freire

; Arrais, Celso

; Lobo, Clarisse Lopes de Castro

; Figueiredo, Maria Stella

; Gualandro, Sandra Fátima Menosi

; Saad, Sara Teresinha Olalla

; Costa, Fernando Ferreira

Hematology, Transfusion and Cell Therapy

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Set 2021, Volume 43 Nº 3 Páginas 341 - 348

Resumo: En | Texto: En | PDF: En

Paroxysmal nocturnal hemoglobinuria is a chronic, multi-systemic, progressive and lifethreatening disease characterized by intravascular hemolysis, thrombotic events, serious infections and bone marrow failure. Paroxysmal nocturnal hemoglobinuria results from the expansion of a clone of hematopoietic cells that due to an inactivating mutation of the X-linked gene PIG-A are deficient in glycosylphosphatidylinositol-linked proteins. Early diagnosis, using flow cytometry performed on peripheral blood, the gold standard test to confirm the diagnosis of paroxysmal nocturnal hemoglobinuria, is essential for improved patient management and prognosis. The traditional therapy for paroxysmal nocturnal hemoglobinuria includes blood transfusion, anti-thrombosis prophylaxis or allogeneic bone marrow transplantation. The treatment that has recently become available is the complement blockade by the anti-C5 monoclonal antibody eculizumab. In this consensus, we are aiming to review the diagnosis and treatment of the paroxysmal nocturnal hemoglobinuria patients, as well as the early recognition of its systemic complications. These procedures express the opinions of experts and have been based on the best available evidence and international guidelines, with the purpose of increasing benefits and reducing harm to patients.

https://doi.org/10.1016/j.htct.2020.06.006 23 downloads

Mortality in children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil
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Lobo, Clarisse Lopes de Castro

; Nascimento, Emilia Matos do

; Jesus, Leonardo José Carvalho de

; Freitas, Thiago Gotelip de

; Lugon, Jocemir Ronaldo

; Ballas, Samir K.

Hematology, Transfusion and Cell Therapy

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Mar 2018, Volume 40 Nº 1 Páginas 37 - 42

Resumo: En | Texto: En | PDF: En

Abstract Objective: To determine the mortality rate of children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil. Methods: The number of deaths, the mortality rate and the causes of deaths in patients with sickle cell anemia who were treated and followed up at our institution for 15 years were determined and compared to data available for the Brazilian population. Results: The overall number of deaths was 281 patients with a mortality rate of 16.77%. Survival probability was significantly higher in females. The number of deaths and the mortality rate were age-specific with a significant increase in the 19- to 29-year-old age group. The remaining life expectancy of the patients with sickle cell anemia was less than that of Brazilians at large. The gap between the two was about 20 years for ages between one and five years with this gap decreasing to ten years after the age of 65 years. The most common causes of death were infection, acute chest syndrome, overt stroke, organ damage and sudden death during painful crises. Conclusion: To the best of our knowledge, this is the first Brazilian study in a single institution in Rio de Janeiro; the mortality rate was 18.87% among adult patients with sickle cell anemia. The mortality rates in children and adults are higher than those reported in developed countries of the northern hemisphere.

https://doi.org/10.1016/j.bjhh.2017.09.006 1485 downloads

Guidelines on the treatment of anemia of chronic renal failure using recombinant human erythropoietin: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines Project: Associação Médica Brasileira – 2014
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Araújo, Aderson da Silva

; Lobo, Clarisse Lopes de Castro

; Covas, Dimas Tadeu

; Costa, Fernando Ferreira

; Medeiros, Letícia

; Cançado, Rodolfo Delfini

; Gualandro, Sandra Fátima Menosi

; Saad, Sara Teresinha Olalla

Revista Brasileira de Hematologia e Hemoterapia

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Dez 2014, Volume 36 Nº 6 Páginas 450 - 453

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1016/j.bjhh.2014.09.009 1896 downloads

Optimization of experimental parameters in preparing multinanoporous TiO2 thin films by the anodic oxidation method
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Ekoko, Gracien B.

; Lobo, Joseph K-K.

; Mvele, Omer M.

; Mbongo, Antoine K.

; Muswema, Jérémie L.

; Lefuni, Clarisse Z.

South African Journal of Chemistry

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Ago 2013, Volume 66

Resumo: En | Texto: En | PDF: En

The anodic oxidation method has been applied to the preparation of multinanoporous TiO2 thin films. The experimental parameters, including the electrolyte nature, oxidation voltage, and oxidation time have been carefully controlled. Their influence on the structure, morphology and photocatalytic activity of the prepared TiO2 films has been evaluated by measuring the current density. The result showed that there was a relatively wide range of preparation conditions, and the internal relationship between the structure and the photocatalytic properties of the TiO2 films was analyzed.

1847 downloads

Brazilian Thalassemia Association protocol for iron chelation therapy in patients under regular transfusion
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Verissimo, Monica Pinheiro de Almeida

; Loggetto, Sandra Regina

; Fabron Junior, Antonio

; Baldanzi, Giorgio Roberto

; Hamerschlak, Nelson

; Fernandes, Juliano Lara

; Araujo, Aderson da Silva

; Lobo, Clarisse Lopes de Castro

; Fertrin, Kleber Yotsumoto

; Berdoukas, Vasilios Antonios

; Galanello, Renzo

Revista Brasileira de Hematologia e Hemoterapia

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2013, Volume 35 Nº 6 Páginas 428 - 434

Resumo: En | Texto: En | PDF: En

In the absence of an iron chelating agent, patients with beta-thalassemia on regular transfusions present complications of transfusion-related iron overload. Without iron chelation therapy, heart disease is the major cause of death; however, hepatic and endocrine complications also occur. Currently there are three iron chelating agents available for continuous use in patients with thalassemia on regular transfusions (desferrioxamine, deferiprone, and deferasirox) providing good results in reducing cardiac, hepatic and endocrine toxicity. These practice guidelines, prepared by the Scientific Committee of Associação Brasileira de Thalassemia (ABRASTA), presents a review of the literature regarding iron overload assessment (by imaging and laboratory exams) and the role of T2* magnetic resonance imaging (MRI) to control iron overload and iron chelation therapy, with evidence-based recommendations for each clinical situation. Based on this review, the authors propose an iron chelation protocol for patients with thalassemia under regular transfusions.

https://doi.org/10.5581/1516-8484.20130106 5074 downloads

Beta-S globin haplotypes in patients with sickle cell anemia: one approach to understand the diversity in Brazil
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Okumura, Jéssika Viviani

; Lobo, Clarisse Lopes de Castro

; Bonini-Domingos, Claudia Regina

Revista Brasileira de Hematologia e Hemoterapia

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2013, Volume 35 Nº 1 Páginas 71 - 72

Resumo: En | Texto: En | PDF: En

https://doi.org/10.5581/1516-8484.20130019 1253 downloads Citado 3 vezes em SciELO

10.

Abnormal transcranial Döppler ultrasonography in children with sickle cell disease
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Leite, Ana Claudia Celestino Bezerra

; Oliveira, Raquel Vasconcellos Carvalhaes de

; Moura, Patrícia Gomes de

; Silva, Célia Maria

; Lobo, Clarisse

Revista Brasileira de Hematologia e Hemoterapia

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2012, Volume 34 Nº 4 Páginas 307 - 310

Resumo: En | Texto: En | PDF: En

BACKGROUND: Stroke is a potentially fatal complication of sickle cell disease in children between 2-16 years and transcranial Döppler has been recommended as a screening method in these cases. OBJECTIVE: The main goal of this study was to correlate transcranial Döppler results to complications related to stroke in sickle cell disease and baseline characteristics of the population. METHODS: This was an observational study of children and adolescents with ages between 2-16 years with sickle cell disease who were followed in three centers. RESULTS: From January 2008 to July 2009, 902 patients were enrolled in this study. The median age was 6.5 years (range: 1.8-15.8), 52.3% were male, 74.4% had hemoglobin SS; 221 (28.6%) had at least one complication associated with sickle cell disease. A total of 773 patients performed transcranial Döppler; in 91.2% this was a method of screening. Conditional or abnormal transcranial Döppler results were more common in patients with sickle cell disease complications versus those without complications (ODDS ratio = 3.18; 95% Confidence interval = 1.92-5.27). There was a significant difference in the frequency of conditional or abnormal transcranial Döppler results in patients with abnormal laboratory results compared to those without abnormalities (OR=4.03); 95% confidence interval = 2.30-7.06. CONCLUSIONS: Conditional or abnormal transcranial Döppler results were significantly more frequent in patients with complications of sickle cell disease confirming the increased risk of stroke in this subgroup of patients. This observation reinforces the recommendation of transcranial Döppler as a screening test for all patients with sickle cell disease with ages between 2 and 16 years.

https://doi.org/10.5581/1516-8484.20120078 1636 downloads

11.

The influence of hydroxyurea on oxidative stress in sickle cell anemia
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Torres, Lidiane de Souza

; Silva, Danilo Grünig Humberto da

; Belini Junior, Edis

; Almeida, Eduardo Alves de

; Lobo, Clarisse Lopes de Castro

; Cançado, Rodolfo Delfini

; Ruiz, Milton Artur

; Bonini-Domingos, Claudia Regina

Revista Brasileira de Hematologia e Hemoterapia

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2012, Volume 34 Nº 6 Páginas 421 - 425

Resumo: En | Texto: En | PDF: En

OBJECTIVE: The oxidative stress in 20 sickle cell anemia patients taking hydroxyurea and 13 sickle cell anemia patients who did not take hydroxyurea was compared with a control group of 96 individuals without any hemoglobinopathy. METHODS: Oxidative stress was assessed by thiobarbituric acid reactive species production, the Trolox-equivalent antioxidant capacity and plasma glutathione levels. RESULTS: Thiobarbituric acid reactive species values were higher in patients without specific medication, followed by patients taking hydroxyurea and the Control Group (p < 0.0001). The antioxidant capacity was higher in patients taking hydroxyurea and lower in the Control Group (p = 0.0002 for Trolox-equivalent antioxidant capacity and p < 0.0292 for plasma glutathione). Thiobarbituric acid reactive species levels were correlated with higher hemoglobin S levels (r = 0.55; p = 0.0040) and lower hemoglobin F concentrations(r = -0.52; p = 0.0067). On the other hand, plasma glutathione levels were negatively correlated with hemoglobin S levels (r = -0.49; p = 0.0111) and positively associated with hemoglobin F values (r = 0.56; p = 0.0031). CONCLUSION: Sickle cell anemia patients have high oxidative stress and, conversely, increased antioxidant activity. The increase in hemoglobin F levels provided by hydroxyurea and its antioxidant action may explain the reduction in lipid peroxidation and increased antioxidant defenses in these individuals.

https://doi.org/10.5581/1516-8484.20120106 2662 downloads Citado 1 vez em SciELO

12.

Brazilian Guidelines for transcranial doppler in children and adolescents with sickle cell disease
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Lobo, Clarisse Lopes de Castro

; Cançado, Rodolfo Delfini

; Leite, Ana Claudia Celestino Bezerra

; Anjos, Ana Claudia Mendonça dos

; Pinto, Ana Cristina Silva

; Matta, Andre Palma da Cunha

; Silva, Célia Maria

; Silva, Gisele Sampaio

; Friedrisch, João Ricardo

; Braga, Josefina Aparecida Pellegrini

; Lange, Marcos Christiano

; Figueiredo, Maria Stella

; Rugani, Marília Álvares

; Veloso, Orlando

; Moura, Patrícia Gomes

; Cortez, Paulo Ivo

; Adams, Robert

; Gualandro, Sandra Fátima Menosi

; Castilho, Shirley Lopes de

; Thomé, Ursula

; Zetola, Viviane Flumignan

Revista Brasileira de Hematologia e Hemoterapia

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Fev 2011, Volume 33 Nº 1 Páginas 43 - 48

Resumo: En | Texto: En | PDF: En

BACKGROUND: Sickle cell disease is the most common monogenic hereditary disease in Brazil. Although strokes are one of the main causes of morbidity and mortality in these patients, the use of transcranial Doppler to identify children at risk is not universally used. OBJECTIVE: To develop Brazilian guidelines for the use of transcranial Doppler in sickle cell disease children and adolescents, so that related health policies can be expanded, and thus contribute to reduce morbidity and mortality. METHODS: The guidelines were formulated in a consensus meeting of experts in transcranial Doppler and sickle cell disease. The issues discussed were previously formulated and scientific articles in databases (MEDLINE, SciELO and Cochrane) were carefully analyzed. The consensus for each question was obtained by a vote of experts on the specific theme. RESULTS: Recommendations were made, including indications for the use of transcranial Doppler according to the sickle cell disease genotype and patients age; the necessary conditions to perform the exam and its periodicity depending on exam results; the criteria for the indication of blood transfusions and iron chelation therapy; the indication of hydroxyurea; and the therapeutic approach in cases of conditional transcranial Doppler. CONCLUSION: The Brazilian guidelines on the use of transcranial doppler in sickle cell disease patients may reduce the risk of strokes, and thus reduce the morbidity and mortality and improve the quality of life of sickle cell disease patients.

https://doi.org/10.5581/1516-8484.20110014 4463 downloads

13.

Tratamento da anemia ferropriva com ferro por via parenteral
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Cançado, Rodolfo D.

; Lobo, Clarisse

; Friedrich, João Ricardo

Revista Brasileira de Hematologia e Hemoterapia

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Jun 2010, Volume 32 Supl. 2 Páginas 121 - 128

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

Embora o ferro por via oral seja considerado a primeira opção de tratamento da deficiência de ferro, em algumas situações específicas, a administração de ferro por via parenteral é uma opção terapêutica que deve ser considerada. Diferentemente do ferro dextran de alto peso molecular utilizado na década de 80 e lembrado como um composto associado ao alto risco de reação anafilática e morte, o desenvolvimento e comercialização de novos compostos com ferro para uso parenteral, sobretudo por via endovenosa - como o ferro sacarato, ferro gluconato e, mais recentemente, a carboximaltose férrica - , tem se tornado cada vez mais uma alternativa terapêutica segura e efetiva, e tem possibilitado ampliar o leque de indicações desta modalidade de tratamento além da nefrologia, como obstetrícia e ginecologia, cirurgia, pediatria, gastroenterologia, hematologia e hemoterapia. Os autores revisam as principais indicações do tratamento com ferro por via parenteral, analisam as principais drogas disponíveis para a correção da anemia ferropriva por via endovenosa e propõem uma estratégia de investigação diagnóstica, tratamento e seguimento laboratorial dos pacientes com indicação desta opção terapêutica.

Although oral iron is generally considered the first choice in the treatment of iron deficiency, in some specific situations, parenteral iron administration is a therapeutic option that should be considered. Different to the high-molecular-weight iron dextran utilized in the eighties and remembered as a compound associated with a high risk of anaphylaxis and death, the development and marketing of newer preparations for parenteral, in particular endovenous, administration, such as iron sucrose, ferric gluconate and more recently ferric carboxymaltose, are becoming a more effective and safe therapeutic alternative, that have extended the range of indications beyond nephrology to obstetrics and gynecology, surgery, pediatrics, gastroenterology, hematology and hemotherapy. The authors review the main indications of parenteral iron treatment, analyse the drugs available for the correction of iron deficiency anemia by intravenous iron administration and propose a new strategy of diagnostic investigation, treatment and laboratory follow up of the patient with indication for this therapeutic option.

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14.

Tratamento da anemia ferropriva com ferro por via oral
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Cançado, Rodolfo D.

; Lobo, Clarisse

; Friedrich, João Ricardo

Revista Brasileira de Hematologia e Hemoterapia

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Jun 2010, Volume 32 Supl. 2 Páginas 114 - 120

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

A anemia ferropriva permanece como uma das deficiências nutricionais mais frequentes e importantes no mundo. O tratamento com ferro deve ser iniciado preferencialmente por via oral e a investigação apropriada de sua causa é obrigatória. Os autores discutem os compostos com ferro atualmente disponíveis, o perfil de eficácia, segurança e tolerabilidade desses medicamentos, e o plano terapêutico mais adequado possível para o sucesso no tratamento dessa doença tão comum e importante.

Iron deficiency anemia remains one of the commonest and most important nutritional deficiencies in the world today. The treatment of iron deficiency should preferably be initiated with oral iron; an appropriate investigation of the cause of iron deficiency anemia is mandatory. The authors discuss the currently available iron preparations as well as their efficacy, safety and tolerability and the optimal therapeutic plan to successfully treat this common and important disease.

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15.

Doença falciforme - um grave problema de saúde pública mundial
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Lobo, Clarisse

Revista Brasileira de Hematologia e Hemoterapia

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2010, Volume 32 Nº 4 Páginas 280 - 281

Resumo: Pt | Texto: Pt | PDF: Pt

5442 downloads Citado 1 vez em SciELO

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