Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio

Rio de Janeiro, Brazil

Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio

Rio de Janeiro, Brazil
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Torres L.S.,São Paulo State University | da Silva D.G.H.,São Paulo State University | Belini Junior E.,São Paulo State University | de Almeida E.A.,São Paulo State University | And 3 more authors.
Revista Brasileira de Hematologia e Hemoterapia | Year: 2012

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.


PubMed | Federal University of Fluminense, Thomas Jefferson University, University Claude Bernard Lyon 1, Centro Universitario Estadual da Zona Oeste and Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio
Type: Journal Article | Journal: PloS one | Year: 2015

This study was a prospective cross-sectional cohort study of 125 patients with sickle cell anemia (SS) between the ages of 16 to 60 years. Enrolled patients were followed-up prospectively for 15 months. Demographic, clinical, hematological and routine biochemical data were obtained on all patients. Six-minute walk test and Doppler Echocardiography were performed on all patients. A tricuspid regurgitant jet velocity (TRJV) < 2.5 m/sec was considered normal, 2.5 TRJV 3.0 was considered mild-moderate and > 3.0 m/sec, severe. Patients with abnormal TRJV were significantly older and more anemic, had significantly higher lactate dehydrogenase (LDH) levels, reticulocyte count and incidence of death. The logistic multimodal model implemented for the 125 patients indicated that age was the covariate that influenced the outcome of normal or abnormal TRJV with a cutoff age of thirty-two years. The survival rate for the group of patients with creatinine (Cr) > 1.0 mg/dL was lower than the group with Cr 1 and normal TRJV. A coefficient matrix showed that the LDH values were weakly correlated with the reticulocyte count but strongly correlated with hemoglobin suggesting that the TRJV values were not correlated with the hemolytic rate but with anemia. Ten patients died during the follow-up of whom 7 had TRJV > 2.5 m/sec. Acute chest syndrome was the most common cause of death followed by sepsis. In conclusion, this study shows that patients with SS older than thirty-two years with high LDH, elevated TRJV, severe anemia and Cr > 1 have poor prognosis and may be at risk of having pulmonary hypertension and should undergo RHC.


de Oliveira Boechat T.,Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio | do Nascimento E.M.,Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio | do Nascimento E.M.,Centro Universitario Estadual da Zona Oeste | de Castro Lobo C.L.,Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio | And 2 more authors.
Pediatric Blood and Cancer | Year: 2015

Background: Deep venous thrombosis (DVT) is rare in children compared to adults. Its incidence and risk factors in children are not well known. This study determined these aspects of DVT in children with sickle cell disease (SCD). Procedure: A retrospective, observational and descriptive study was performed. Patients born between October 2000 and October 2012 with SCD and registered in HEMORIO, including those who died in HEMORIO, were included in this study. Patients whose medical records were inaccessible, who died in institutions other than HEMORIO, who died with implanted deep venous catheters, and those who were not monitored in HEMORIO for a period of 1 year or more were excluded from the study. Of a total of 1,519 patients, 456 were excluded and 1,063 patients were included in the study. Data were obtained from the computer system and the medical records at HEMORIO. Results: Of the 1,063 patients, 2 (0.2%) developed DVT with both cases being related to central venous catheters (CVCs) (P-value <0.001). Of the patients who required CVCs, the prevalence of DVT was 10%. No other variable was clinically or statistically significant with respect to DVT. Conclusion: The establishment of CVCs in children with SCD poses a high risk for DVT. If this procedure is necessary, the internal jugular vein should be utilized instead of the subclavian and femoral veins. The identification of associated risk factors may justify antithrombotic prophylaxis. Pediatr Blood Cancer 2015;62:838-841. © 2015 Wiley Periodicals, Inc.


Lobo C.L.C.,Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio | Ballas S.K.,Thomas Jefferson University | Domingos A.C.B.,Institute Hematologia Arthur Of Siqueira | Moura P.G.,Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio | And 4 more authors.
Pediatric Blood and Cancer | Year: 2014

Background: Newborn screening for hemoglobinopathy in Brazil has been decentralized until 2001 when the Health Ministry of Brazil established the National Newborn Hemoglobinopathy Screening Program. The State of Rio de Janeiro started a program in collaboration with the State Health Department and the Institute of Hematology in Rio (HEMORIO). The goal of this study was to evaluate the effectiveness of the first 10 years of the Newborn Hemoglobinopathy Screening Program in identifying and managing infants with Sickle cell disease (SCD) in the State of Rio de Janeiro. Procedure: Blood samples from 1,217,833 neonates were analyzed by High Performance Liquid Chromatography. Infants with SCD were enrolled in comprehensive treatment programs. Results: Data showed that 4.87% of the newborns were heterozygous for a hemoglobin variant, 0.08% were homozygous or doubly heterozygous for abnormal hemoglobins and 95.02% had normal hemoglobin. All the 912 newborns with SCD were referred for treatment at HEMORIO, 34 (3.7%) of these died due to acute chest syndrome, sepsis or splenic sequestration. Four more children died of unknown causes. The implementation of the Rio de Janeiro Newborn Screening Program gradually increased the area of the State covered by the program. Conclusion: Data collected during the 10 years of the program showed reduction in mortality of patients with SCD in comparison to available historical statistical data before the implementation of the national screening program. This 10-year study showed that early diagnosis and treatment of newborns was associated with improved survival and quality of life of Brazilian children with SCD. © 2013 Wiley Periodicals, Inc.


Queiroz A.M.M.,Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio | Campos J.,Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio | Lobo C.,Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio | Bonini-Domingos C.R.,São Paulo State University | And 2 more authors.
Hemoglobin | Year: 2014

A 35-year-old African Brazilian patient had sickle cell anemia complicated with recurrent vasoocclusive (VOC) crises and refractory painful leg ulcers for 16 years. The ulcers started over both medial malleoli and expanded gradually. The ulcer on the left leg spread from the foot to the knee circumferentially and was refractory to all forms of therapy within the frame work of multi-disciplinary care. The patient agreed to a below the knee amputation of the left leg. He felt much better after the amputation but developed severe neuropathic phantom pain that was well controlled medically. He could differentiate the sickle cell anemia and ulcer pain from the neuropathic pain. About 6 months after the amputation he had dengue fever with fatal outcome. This is the first report of treatment of refractory sickle cell anemia leg ulcer with amputation and probably the first report of a Brazilian patient with sickle cell anemia and dengue fever. © 2014 Informa Healthcare USA, Inc. All rights reserved: reproduction in whole or part not permitted.


Fernandes L.L.D.,Federal University of Rio de Janeiro | Torres S.R.,Federal University of Rio de Janeiro | Garnica M.,Federal University of Rio de Janeiro | De Souza Goncalves L.,Federal University of Rio de Janeiro | And 5 more authors.
Supportive Care in Cancer | Year: 2014

Purpose: Oral infection may be a source of bacteremia in patients undergoing hematopoietic stem cell transplant (HSCT). The aim of this study was to evaluate the relationship between patients with poor periodontal status and complications after HSCT. Methods: A cohort of patients with hematological malignancies candidates for autologous HSCT was observed before and during the neutropenic phase of HSCT. A primary evaluation was performed before the HSCT procedure, including medical and socio-demographic data and physical examination (number of teeth and decayed, missing and filled teeth index (DMFT), oral mucosa, and full mouth periodontal assessment). During the neutropenic phase, data regarding the development of febrile neutropenia, bacteremia, and mucositis were also prospectively obtained. Results: Forty-eight patients were included. The most common baseline disease was multiple myeloma (70 %). In the primary evaluations, the median DMFT was 13 (ranging 0-27), and periodontitis and gingivitis were present in 29 and 60 % of the patients, respectively. During the neutropenic phase of HSCT, fever occurred in 96 % of patients, and bacteremia was documented in 29 %. Coagulase-negative Staphylococcus was the most common isolated bacteria. Patients who developed bacteremia had a higher frequency of oral disorders compared with those without bacteremia, but it was not statistically significant. Oral mucositis affected 89.6 % of the patients, and patients with gingivitis or periodontal disorders had a high frequency of mucositis. Conclusions: The prevalence of oral pathologic conditions previous to HSCT procedures was very high in the studied population. A possible association was noted between previous gingivitis and the development of mucositis during the neutropenia of HSCT. © 2013 Springer-Verlag Berlin Heidelberg.


PubMed | Institute Arthur Of Siqueira Cavalcanti Hemorio, Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio and University of Campinas
Type: Journal Article | Journal: Hemoglobin | Year: 2016

In this study, we describe four new patients with sickle cell disease who had limb amputations. Two of the patients had sickle cell anemia [Hb S (HBB: c.20A>T) ((S)/(S))] with refractory leg ulcers that required amputations. The third patient had sickle cell trait with an extensive leg ulcer that was associated with epidermoid carcinoma. The fourth patient had amputations of both forearms and feet due to a misdiagnosis of dactylitis. Review of the literature showed that the indications for amputations in sickle cell disease included three distinct categories: mythical beliefs, therapeutic and malpractice. All therapeutic amputations were for severely painful, large, recalcitrant leg ulcers that failed non-interventional therapies. Amputation resulted in pain relief and better quality of life. Phantom neuropathic pain was not a major issue post-operatively. It was absent, transient or well controlled with antidepressants. Limb function was restored post-amputation with prosthetic artificial limbs, wheelchairs or crutches. Malpractice amputations were due to misdiagnosis or to cryotherapy by exposing the painful limb to ice water resulting in thrombosis, gangrene and amputation. We strongly suggest that leg amputations should be considered in the management of certain patients with severe extensive refractory leg ulcers, and topical cryotherapy should never be used to manage sickle cell pain.


PubMed | Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio
Type: Journal Article | Journal: Pediatric blood & cancer | Year: 2013

Newborn screening for hemoglobinopathy in Brazil has been decentralized until 2001 when the Health Ministry of Brazil established the National Newborn Hemoglobinopathy Screening Program. The State of Rio de Janeiro started a program in collaboration with the State Health Department and the Institute of Hematology in Rio (HEMORIO). The goal of this study was to evaluate the effectiveness of the first 10 years of the Newborn Hemoglobinopathy Screening Program in identifying and managing infants with Sickle cell disease (SCD) in the State of Rio de Janeiro.Blood samples from 1,217,833 neonates were analyzed by High Performance Liquid Chromatography. Infants with SCD were enrolled in comprehensive treatment programs.Data showed that 4.87% of the newborns were heterozygous for a hemoglobin variant, 0.08% were homozygous or doubly heterozygous for abnormal hemoglobins and 95.02% had normal hemoglobin. All the 912 newborns with SCD were referred for treatment at HEMORIO, 34 (3.7%) of these died due to acute chest syndrome, sepsis or splenic sequestration. Four more children died of unknown causes. The implementation of the Rio de Janeiro Newborn Screening Program gradually increased the area of the State covered by the program.Data collected during the 10 years of the program showed reduction in mortality of patients with SCD in comparison to available historical statistical data before the implementation of the national screening program. This 10-year study showed that early diagnosis and treatment of newborns was associated with improved survival and quality of life of Brazilian children with SCD.


PubMed | Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio
Type: Journal Article | Journal: European journal of haematology | Year: 2014

Describe the treatment of patients with vaso-occlusive crises (VOC) in a Brazilian emergency department (ED) and the successful switch from intravenous to oral morphine.We analyzed records of 315 patients with sickle cell disease using two different protocols for pain: one in March 2010 prescribing intravenous morphine every 4 h throughout their stay, and another in March 2011 and 2012 prescribing one initial dose of intravenous morphine followed by equianalgesic doses of oral morphine every 4 h. Patients were triaged into three groups: mild, moderate, and severe VOC. The mild group was treated within 1 h after triage, the moderate within 30 min and the severe was treated immediately. Patients whose pain was not relieved within 6 h after the first dose of morphine were transferred into a different holding area of the ED where they continued to receive the same treatment for 48 h after which they were hospitalized if still in pain.The number of patients who stayed <24 h in the ED increased significantly from 63 in 2010 to 87 in 2012, and the number of admissions decreased from 26 in 2010 to 10 in 2012. The incidence of acute chest syndrome decreased from 8.5% in 2010 to 1.9% in 2012.Patients treated with oral morphine stayed a shorter time in the ED, had more pain relief, were admitted less frequently, and had less acute chest syndrome. These differences may be due to environmental, cultural, psychological, and pharmacogenetic factors.


PubMed | Institute Hematologia Arthur Of Siqueira Cavalcanti Hemorio
Type: Journal Article | Journal: Pediatric blood & cancer | Year: 2015

Deep venous thrombosis (DVT) is rare in children compared to adults. Its incidence and risk factors in children are not well known. This study determined these aspects of DVT in children with sickle cell disease (SCD).A retrospective, observational and descriptive study was performed. Patients born between October 2000 and October 2012 with SCD and registered in HEMORIO, including those who died in HEMORIO, were included in this study. Patients whose medical records were inaccessible, who died in institutions other than HEMORIO, who died with implanted deep venous catheters, and those who were not monitored in HEMORIO for a period of 1 year or more were excluded from the study. Of a total of 1,519 patients, 456 were excluded and 1,063 patients were included in the study. Data were obtained from the computer system and the medical records at HEMORIO.Of the 1,063 patients, 2 (0.2%) developed DVT with both cases being related to central venous catheters (CVCs) (P-value <0.001). Of the patients who required CVCs, the prevalence of DVT was 10%. No other variable was clinically or statistically significant with respect to DVT.The establishment of CVCs in children with SCD poses a high risk for DVT. If this procedure is necessary, the internal jugular vein should be utilized instead of the subclavian and femoral veins. The identification of associated risk factors may justify antithrombotic prophylaxis.

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