Asthma, allergic sensitization and lung function in sickle cell disease.

BACKGROUND: Pulmonary disease is a frequent acute and chronic manifestation in sickle cell disease (SCD), presenting high morbidity and mortality. OBJECTIVES: To identify the prevalence and association of asthma, allergic sensitization and altered pulmonary function in patients with SCD (SS and Sßo). METHODS: A single-center, cross-sectional study was conducted, in which 70 patients with SCD and 44 controls, aged six to 18 years, responded to the questionnaire of the International Study of Asthma and Allergies in Childhood (ISAAC), complemented with an anamnesis regarding the associated clinical outcomes. All patients underwent immediate hypersensitivity skin tests with aeroallergens and a pulmonary function evaluation (spirometry). Regarding the statistical analysis, parametric and non-parametric methods were used, depending on the variables studied. Tests were considered significant when p<0.05. RESULTS: There was no significant difference between the patients and controls regarding the prevalence of asthma and allergic sensitization (p>0.05). The number of occurrences of acute chest syndrome per patient per year was significantly higher for asthmatic patients than for non-asthmatic patients (p=0.04). Obstructive pulmonary function occurred in 30.9% of the patients and in 5.4% of the controls, and restrictive pulmonary function occurred in 5.5% of the patients and 5.4% of the controls. Asthma and wheezing in the last 12months had significant associations with obstructive pulmonary function (p=0.014 and p=0.027, respectively). CONCLUSIONS: The occurrence of asthma, allergic sensitization and alteration in lung function in patients with SCD reinforces the importance of routine monitoring of these diagnoses, which allows for early treatment and prevention of the evolution of pulmonary disease in adulthood.

Lung Function in Infants with Sickle Cell Anemia.

Lung volumes and forced expiratory flows were evaluated in 22 infants with sickle cell anemia and compared with a control group. Forced expiratory flows showed significantly lower values in the sickle cell group. The majority of infants had normal lung function, and obstruction was the most common pattern of abnormality.

Cerebral Vasoreactivity in Children with Sickle Cell Disease: A Transcranial Doppler Study.

BACKGROUND: Impairment of vasodilatory capacity reflecting reduced cerebrovascular reserve was previously shown in adults with sickle cell disease (SCD) and might play a role in the pathophysiology of stroke in such patients. We examined the hypothesis that children with SCD would also have a higher frequency of impaired cerebral vasoreactivity when compared with healthy age- and gender-matched controls. METHODS: Patients were recruited from our hematology outpatient clinic. All SCD patients aged 10-18 years without a history of symptomatic stroke as well as age- and gender-matched healthy children were evaluated with transcranial Doppler (TCD) ultrasonography, with breath-holding maneuver. Breath-holding index (BHI) was calculated by dividing the percentage increase in mean flow velocity occurring during breath holding by the length of time subjects hold their breath after a normal inspiration. BHI was considered abnormal if less than .69. RESULTS: TCD was performed in 42 patients (mean age 12.7 ± 2.2 years) and 20 controls (mean age 13.90 ± 3.04 years). Blood flow velocities were higher in patients with SCD than in controls in all arteries evaluated (P < .001). BHI values in patients with SCD were significantly lower than in control subjects (1.27 ± .65 versus 1.74 ± .15, P = .013 on the left and 1.16 ± .45 versus 1.61 ± .11, P = .002 on the right). BHI was abnormal in 19% of the patients and in none of the controls, P = .036. CONCLUSIONS: Children with SCD may have impaired cerebral vasoreactivity, with low BHI values suggesting a reduced autoregulation capacity.

Changes in Transcranial Doppler Flow Velocities in Children with Sickle Cell Disease: The Impact of Hydroxyurea Therapy.

BACKGROUND AND OBJECTIVES: Hydroxyurea (HU) was recently described as a substitute for chronic transfusion for children with sickle cell disease (SCD) and abnormal transcranial Doppler (TCD) velocities who have received at least 1 year of transfusions. However, the role of HU in reverting elevated TCD velocities in patients not treated with transfusion is still debatable. The objective of the study was to examine whether HU influences the progression of TCD velocities in children with SCD. PATIENTS AND METHODS: Children with SCD with at least 2 TCDs not less than 6 months apart were evaluated over 51 months. Time-averaged maximum mean (TAMM) velocities for the initial and the last transcranial Doppler examinations were noted and differences compared between HU and HU-naive groups. RESULTS: Overall, 68.8% of the HU-group with elevated TCD velocities compared with 40.0% of the HU-naive experienced TCD reversal (P = .047). A higher proportion of the HU-naive group, 7 (14.3%) versus 9.8% of the HU group experienced TCD conversion. Those with initial conditional velocities in the HU-group experienced a significant reduction in TAMM velocities (from 176.8 ± 5.3 to 162.7 ± 13.9 cm/s, difference of 14.1 cm/s; P = .001) unlike those in the HU-naive group (176.3 ± 5.3 to 170.0 ± 18.6 cm/s, difference of 6.3 cm/s; P = .148). The change in the TAMM velocities was also significantly higher among the HU-group (14.1 ± 12.4 cm/s versus 6.3 ± 18.5 cm/s, P = .015). CONCLUSION: Our data suggest a beneficial role of HU in TCD velocity reduction in patients not treated with chronic transfusions, particularly among those with initial conditional TCD velocities.

Helicobacter pylori infection & immune thrombocytopenic purpura in children and adolescents: A randomized controlled trial.

Helicobacter pylori and immune thrombocytopenic purpura (ITP) association is not well established in chronic ITP (cITP) in children, although the cure of thrombocytopenia in approximately half of H. pylori eradicated adult patients has been described. The aim of this study was to investigate the effect of H. pylori eradication on platelet (PLT) recovery in cITP children and adolescents through a randomized, controlled trial. A total of 85 children (mean age 11.4 years) with cITP were prospectively enrolled. Diagnosis of H. pylori was established by two locally validated tests, (13)C-urea breath test and monoclonal stool antigen test. Twenty-two infected patients were identified, and randomly allocated into two groups: H. pylori treatment group (n = 11) and the non-intervention control group (n = 11). The control group was offered treatment if the thrombocytopenia persisted after the follow-up. At baseline, there were no differences regarding age, sex, duration of disease, and PLT count between groups. Sixty three of 85 patients were uninfected. PLT response was classified as complete response: PLT > 150 × 10(9 )l(-1); partial response: PLT 50-150 × 10(9 )l(-1), or an increase of 20-30 × 10(9 )l(-1); no response: PLT < 50 × 10(9 )l(-1) or an increase of <20 × 10(9 )l(-1) after at least 6 months of follow-up. Complete response was observed in 60.0% (6/10, one excluded) H. pylori eradicated patients vs. 18.2% (2/11) in non-eradicated patients (p = 0.08; OR = 6.75) after 6-9 months of follow-up. Among uninfected patients, only 13.8% (8/58) presented complete response. Two non-treated controls were treated after 6-12 months of follow-up, and PLT response was observed in 61.5% (8/13) of H. pylori eradicated patients, and in 19.0% (11/58) of uninfected patients (p = 0.004). Cytotoxin associated gene A and vacuolating cytotoxin gene A IgG antibodies were present in almost all infected patients. Therefore, the study suggests that H. pylori eradication plays a role in the management of H. pylori infected cITP children and adolescents.

Thrombotic microangiopathy in patients with sickle cell disease.

OBJECTIVE: To describe two cases of patients who had thrombotic microangiopathy (TMA) associated with sickle cell disease (SCD). CASE DESCRIPTION: Both patients started with a painful crisis and had acute chest syndrome during hospitalization. They showed significant worsening of hemolytic anemia, with very high levels of lactate dehydrogenase, thrombocytopenia, lowered level of consciousness, organ damage and the presence of schistocytes in peripheral blood. Due to the possibility of TMA, despite the very rare association with SCD, they were treated with fresh frozen plasma replacement and plasmapheresis, with good response. COMMENTS: TMA is a serious, life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. The association of SCD and TMA is difficult to diagnose, since they can share a similar clinical presentation. Recognizing this association and promptly instituting treatment may impact the survival of these patients.

Mortality trends and sociodemographic factors associated with early death in sickle cell disease patients in the state of São Paulo.

OBJECTIVE: To estimate trends in mortality rate and average age of death, and identify sociodemographic factors associated with early death in patients with sickle cell disease (SCD). METHODS: An ecological and cross-sectional study was conducted using data from the Mortality Information System. All deaths of patients residing in the state of São Paulo from 1996 to 2015 with at least one International Disease Code for SCD in any field of the death certificate were included. Simple linear regression was used to estimate trends. The Log-rank test and multiple Cox regression were used to identify factors associated with early death. RESULTS: The age-standardized mortality rate per million inhabitants increased by 0.080 per year (R2=0.761; p<0.001). When the events were stratified by age at death, the increase was 0.108 per year for those occurring at age 20 years or older, (R2=0.789; p<0.001) and 0.023 per year for those occurring before age 20 years old (R2=0.188; p=0.056). The average age at death increased by 0.617 years (7.4 months) per year (R2=0.835; p<0.001). Sociodemographic factors associated with early death identified were male gender (hazard ratio - HR=1.30), white race (HR=1.16), death occurring in the hospital (HR=1.29), and living in the Greater São Paulo (HR=1.13). CONCLUSIONS: The mortality rate and the average age of death in patients with SCD have increased over the last two decades. Sociodemographic factors such as gender, race, place of occurrence, and residence were found to be associated with early death.

Consensus of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy (ABHH) and the Brazilian Ministry of Health - General management of blood and blood products on the tests necessary for the release of exceptional medicines for sickle cell disease.

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.

Hydroxyurea therapy in sickle cell anemia patients aids to maintain oral fungal colonization balance.

BACKGROUND: The aim of this study was to evaluate the frequency of Candida species and presence of lesions in the oral cavity of patients with sickle cell anemia (SS). METHODS: The study included 30 patients diagnosed with sickle cell anemia and taking hydroxyurea for at least 90 days (SS/HU+); and 39 patients with sickle cell anemia and without hydroxyurea therapy (SS/HU-). Two control groups were constituted by healthy individuals matched to the test groups in age, gender, and oral conditions (C/HU+ for SS/HU+ and C/HU- for SS/HU-). Oral clinical examination and anamnesis were performed. Yeasts were collected by oral rinses and identified by API system. Antifungal susceptibility evaluation was performed according to the CLSI methodology. Data obtained for microorganisms counts were compared by Student's t test (SS/HU+ vs. C/HU+ and SS/HU- vs. C/HU-) using MINITAB for Windows 1.4. Significance level was set at 5%. RESULTS: No oral candidosis lesions were detected. Significant differences in yeasts counts were observed between SS/HU- group and the respective control, but there were no differences between SS/HU+ and C/HU+. Candida albicans was the most prevalent species in all groups. Candida famata was observed both in SS and control groups. Candida dubliniensis, Candida glabrata, Candida krusei, Candida tropicalis, Candida pelliculosa, and Candida parapsilosis were observed only in SS groups. Most strains were susceptible to all antifungal agents. CONCLUSION: Hydroxyurea therapy seems to decrease candidal counts and resistance rate in sickle cell anemia patients. However, further studies should be conducted in the future to confirm this finding. Hydroxyurea therapy in sickle cell anemia patients maintains fungal species balance in oral cavity.

Clinical and laboratory differences between pediatric hospitalized patients with sickle cell disease infected or not by SARS-CoV-2.

OBJECTIVE: The aim of this study was to identify clinical and complete blood count differences between pediatric hospitalized patients with sickle cell disease infected or not by SARS-CoV-2 and compare the complete blood count of patients with sickle cell disease infected by SARS-CoV-2 before hospitalization and on admission. METHODS: This study was a single-center prospective cohort. Data were collected from medical records of pediatric inpatients with sickle cell disease under 18 years old infected or not with SARS-CoV-2 from the first visit to the hospital until discharge and from the last medical appointment. All patients were tested for SARS-CoV-2 by the real-time reverse transcription polymerase chain reaction. RESULTS: Among 57 pediatric patients with sickle cell disease hospitalized from March to November 2020 in a Brazilian academic hospital, 11 (19.3%) had a positive result for SARS-CoV-2. Patients infected by SARS-CoV-2 had a higher prevalence of comorbidities than the ones who were not infected (63.6 vs. 30.4%; p=0.046). During hospital stay, no clinical or complete blood count differences between groups were found. There was a decrease in eosinophil count on hospital admission in patients with sickle cell disease infected by SARS-CoV-2 (p=0.008). CONCLUSIONS: Pediatric hospitalized patients with sickle cell disease infected by SARS-CoV-2 had more comorbidities and had a decrease in eosinophil count between hospital admission and the last medical appointment.

Factors related to the readiness of Brazilian chronic pediatric patients to transition to care in adult clinics.

OBJECTIVE: Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. METHOD: In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. RESULTS: The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. CONCLUSION: TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores.

Use of iron-fortified rice reduces anemia in infants.

Food fortification is advocated to tackle iron deficiency in anemic populations. Our objective was to evaluate the impact of iron-fortified rice (Ultrarice(®)) weekly on hemoglobin and anemia levels compared with standard rice (control). This cluster-randomized study deals with infants (10-23 months) from two public child day care centers in Brazil, n = 216, in an 18 week intervention. The intervention group received individual portions of fortified rice (50 g) provided 56.4 mg elemental/Fe. For intervention center: baseline mean hemoglobin was 11.44 ± 1.07 g/dl, and after intervention 11.67 ± 0.96 g/dl, p < 0.029; for control: baseline mean hemoglobin value was 11.35 ± 4.01 g/dl, and after intervention 11.36 ± 2.10 g/dl, p = 0.986. Anemia prevalence for intervention center was 31.25% at baseline, and 18.75% at end of study, p = 0.045; for control 43.50% were anemic at baseline, and 37.1% at the end of study, p = 0.22. Number Needed to Treat was 7. Iron-fortified rice was effective in increasing hemoglobin levels and reducing anemia in infants.

Milk-based cornstarch porridge fortified with iron is effective in reducing anemia: a randomized, double-blind, placebo-controlled trial.

This study evaluates the impact of a milk-based cornstarch porridge fortified with iron, in 4-year olds, compared with control on hemoglobin levels and anemia prevalence. This trial was a cluster-randomized, double-blind one, and used milk-based cornstarch porridge fortified with 10 mg elemental iron (FeSO(4)), daily, during 14 weeks, compared with control. The study population comprised 4-year-old preschoolers (n = 131). Mean hemoglobin values at baseline were found to be 10.6 ± 0.61 g dl(-1) for intervention group, and after intervention 11.5 ± 0.80 g/dl, p < 0.0001. For control, mean hemoglobin values at baseline were 10.9 ± 0.53 g/dl, and after intervention 11.2 ± 0.73, p < 0.0001. The increase in mean hemoglobin was much greater in the intervention than in the control group, 0.86-0.26 g dl(-1), respectively (p < 0.0001). Anemia prevalence reduced from 75% to 20%, p < 0.0001, in the intervention group, with no reduction in the control group. Number needed for treatment was four. This study showed that milk-based cornstarch porridge fortified with ferrous sulfate increased hemoglobin levels and reduced anemia prevalence in 4-year-old preschoolers.

Weekly iron supplementation for the prevention of anemia in pre-school children: a randomized, double-blind, placebo-controlled trial.

OBJECTIVE: This study analyses the impact of weekly iron supplementation with ferrous sulphate heptahydrate (FeSO4) in 5-year-olds compared with placebo, on hemoglobin (Hb) and hematocrit (Ht) values and anemia. DESIGN: The study concerns a cluster-randomized, placebo-controlled double-blind trial. Intervention participants received 50 mg elemental iron for 14 weeks. SETTING: The study population comprised pre-school children (n = 135) from one randomly chosen public school in the northeast of Brazil. SUBJECTS: Participants were 5-year-old students from a public school. RESULTS: Mean Hb and Ht values increased after iron supplementation, with p < 0.0001. There was no statistically significant increase in the placebo group. After intervention, anemia prevalence reduced only in the intervention group, from 48.0% to 26.0%. CONCLUSIONS: Weekly iron supplementation was effective in reducing anemia in 5-year-olds.

Hemoglobinopathy and pediatrics in the time of COVID-19.

INTRODUCTION: It is important to know if patients with hemoglobinopathy could be more susceptible to COVID-19. OBJECTIVE: Analyze SARS-CoV-2 infection in pediatric patients with hemoglobinopathy. METHODS: Using the online platforms LILACS, PUBMED and EMBASE, on 17- JUL-2020 a search was made for the terms COVID-19 and SARS-CoV-2 associated with "sickle cell", "thalassemia" and "hemoglobinopathy". RESULTS: There were 623 pediatric and adult patients with sickle cell disease (SCD) or beta thalassemia (BT) and COVID-19. Total mortality rate was 6.42%. No pediatric patient with BT has been described. So, our analysis focused on children and adolescents with SCD: there were 121 pediatric patients, one adolescent died, prophylactic anticoagulation was prescribed to six patients, 11.76% needed intensive care unit, blood transfusion was prescribed in 29.70%. Vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) were the main clinical manifestations in SCD. DISCUSSION: Pediatric patients with SCD and COVID-19 have a low mortality rate when compared to adults, although is higher than the global pediatric population with COVID-19 (0-0.67%). The comorbidities associated with age and the long-term complications inherent to hemoglobinopathies may contribute to the increased mortality outside the pediatric age group. In SCD the clinical manifestations, both in children and adults, are VOC and ACS, and there was increase in blood requirement. Pediatric SCD patients with COVID-19 need more intensive care unit than the global pediatric population (3.30%). CONCLUSION: Despite pediatric population with SCD needs more intensive care, the outcome after infection by COVID-19 is favorable.

BEHAVIORAL CHARACTERISTICS OF CHILDREN WITH SICKLE CELL DISEASE.

OBJECTIVE: To evaluate sociodemographic and clinical aspects of children with sickle cell disease (SCD) and their behavioral characteristics. METHODS: Interview with parents of patients with SCD from four to ten years old, addressing socioeconomic aspects and other health conditions, and using the Strengths and Difficulties Questionnaire (SDQ). Clinical data were obtained from medical records. Exclusion criteria were the use of hydroxyurea, previous diagnosis of stroke, chronic encephalopathy and/or intellectual disability. RESULTS: 45 patients (19 girls and 26 boys) were assessed. The median age was seven years. Diagnosis of SCD: 26 hemoglobinopathy SC; 19 hemoglobinopathy SS. Socioeconomic class: D: 24.4%; C2: 44.4%; C1: 28.9%; B2: 2.2%. Clinical history: acute chest syndrome: 40%; transfusions: 66.7%; hospitalizations: 82.2%. SDQ findings: 88.9% clinical impact (emotional subscale: 68.9%); total score: impact in 48.9%. It was not possible to establish a relation between the severity of the disease and the results of the SDQ. Regarding socioeconomic class: among individuals of classes B2 and C1, 21.4% had impact at the total score; in classes C2 and D, this percentage was 61.3%. Regarding the schooling of the head of the family, with Elementary School at least, 39.3% of the children had impacts; for fewer education, this percentage was 64.7%. CONCLUSIONS: Behavioral impacts are highly prevalent in children with SCD. Individuals in socioeconomic classes C2 and D suffered more behavioral impacts than individuals in classes B2 and C1.

Levels of angiotensin-converting enzyme 1 and 2 in serum and urine of children with Sickle Cell Disease.

INTRODUCTION: Sickle cell nephropathy begins in childhood and presents early increases in glomerular filtration, which, over the long term, can lead to chronic renal failure. Several diseases have increased circulating and urinary angiotensin-converting enzyme (ACE) activity, but there is little information about changes in ACEs activity in children with sickle cell disease (SCD). OBJECTIVE: We examined circulating and urinary ACE 1 activity in children with SCD. METHODS: This cross-sectional study compared children who were carriers of SCD with children who comprised a control group (CG). Serum and urinary activities of ACE were evaluated, as were biochemical factors, urinary album/creatinine rates, and estimated glomerular filtration rate. RESULTS: Urinary ACE activity was significantly higher in patients with SCD than in healthy children (median 0.01; range 0.00-0.07 vs median 0.00; range 0.00-0.01 mU/mL·creatinine, p < 0.001. No significant difference in serum ACE activities between the SCD and CG groups was observed (median 32.25; range 16.2-59.3 vs median 40.9; range 18.0-53.4) mU/m`L·creatinine, p < 0.05. CONCLUSION: Our data revealed a high urinary ACE 1 activity, different than plasmatic level, in SCD patients suggesting a dissociation between the intrarenal and systemic RAAS. The increase of urinary ACE 1 activity in SCD patients suggests higher levels of Ang II with a predominance of classical RAAS axis, that can induce kidney damage.

Randomized controlled trial of iron-fortified drinking water in preschool children.

OBJECTIVES: To evaluate the effects of fortified drinking water, with different concentrations of iron added, on hemoglobin and hematocrit values in preschoolers. METHODS: Double-blind, randomized cluster clinical trial, with children aged 2 to 5 years of age, from 4 state-run schools, forming 1 group for each school. For fortification, ferrous sulphate in concentrations of 5 mg of elemental iron per liter of water (group A), 7.5 mg (group B), and 10 mg (group C), was used during a period of 4 months. In group D, the control, a placebo (Bixa orellana) was added. Hemoglobin and hematocrit values were checked before and after intervention. RESULTS: Before fortification, hemoglobin and hematocrit averages were below the reference values adopted in all groups. After fortification, the prevalence of anemia showed a reduction in the 4 groups, which was more pronounced in group B, at 48.3%. The hemoglobin values in groups B (11.5) and C (11.4) were statistically similar. However, the average consumption of water/day/student was lower in group C. Comparison of hemoglobin values between groups A (11.2) and D (11.0) did not show a significant difference, suggesting insignificant efficacy with 5 mg Fe/L fortification. CONCLUSIONS: The consumption of drinking water fortified with 7.5 mg of elemental iron/L water resulted in greater adhesion and an increase in hemoglobin values, with a reduction in the prevalence of anemia.

Chronic transfusion therapy effectiveness as primary stroke prophylaxis in sickle cell disease patients.

INTRODUCTION: About 10% of sickle cell anemia patients will have ischemic stroke. Adams showed stroke incidence reduction in children receiving monthly erythrocyte transfusions by reducing transcranial Doppler (TCD) velocities. Since then, chronic transfusion is recommended as primary stroke prophylaxis. This study aims to assess the effectiveness of chronic transfusions as stroke prophylaxis. METHOD: Retrospective study, reviewing medical records from 15 sickle cell anemia patients undergoing chronic transfusion. Data collected were age, sex, adverse reactions, stroke, hemoglobin, reticulocytes, ferritin, HbS and TCD values (baseline, after 12 and 24months of treatment). RESULTS: The mean age was 118.67±41.40 months; six patients experienced allergic reactions. No stroke was recorded. One patient had alloimmunization. There was a decrease in the HbS rate and an increase in hemoglobin values in the first 12months. Values were maintained after 24months, but with no improvement of data. Before treatment, the mean HbS rate was 75.18%±11.69; after 12months, 41.63±14.99 and after 24months, 43.78±10.6. Thirteen patients initiated chelation after 12months from the beginning of chronic transfusions and ferritin decline after 24months. Pre-transfusional TCD velocities were 204.28±9.41cm/s (right) and 198.85±33.37cm/s (left). After a 12-month treatment, these values were 158.5±28.89cm/s and 157.62±34.43cm/s, respectively, and this reduction was statistically significant (p=0.002 right and p=0.02 left). After 24months, these values were 149.63±26.95cm/s (right) and 143.7±32.27cm/s (left). CONCLUSION: Significant reduction of TCD velocity occurred after treatment with chronic transfusion in sickle cell anemia patients, leading to a normal or conditional test and reducing stroke risk in all but one patient.

Thrombotic microangiopathy in patients with sickle cell disease / Microangiopatia trombótica em pacientes com doença falciforme

ABSTRACT Objective: To describe two cases of patients who had thrombotic microangiopathy (TMA) associated with sickle cell disease (SCD). Case description: Both patients started with a painful crisis and had acute chest syndrome during hospitalization. They showed significant worsening of hemolytic anemia, with very high levels of lactate dehydrogenase, thrombocytopenia, lowered level of consciousness, organ damage and the presence of schistocytes in peripheral blood. Due to the possibility of TMA, despite the very rare association with SCD, they were treated with fresh frozen plasma replacement and plasmapheresis, with good response. Comments: TMA is a serious, life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. The association of SCD and TMA is difficult to diagnose, since they can share a similar clinical presentation. Recognizing this association and promptly instituting treatment may impact the survival of these patients.

RESUMO Objetivo: Descrever dois casos de pacientes que apresentaram microangiopatia trombótica (MAT) associada à doença falciforme (DF). Descrição do caso: Ambos os pacientes iniciaram com crise dolorosa e apresentaram síndrome torácica aguda durante a internação. Eles apresentaram piora significativa da anemia hemolítica, com níveis muito elevados de lactato desidrogenase, trombocitopenia, rebaixamento do nível de consciência, lesão de órgãos e presença de esquistócitos no sangue periférico. Diante da possibilidade de MAT, apesar da associação muito rara com DF, eles foram tratados com reposição de plasma fresco congelado e plasmaférese, com boa resposta. Comentários: A MAT é uma doença grave e com risco de vida, caracterizada por anemia hemolítica microangiopática, trombocitopenia e danos a órgãos. A associação de DF e MAT é de difícil diagnóstico, pois as duas podem ter apresentação clínica semelhante, portanto reconhecer essa associação e instituir o tratamento prontamente pode ter grande impacto na sobrevida desses pacientes.