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.

Platelet-rich plasma in diabetic dry eye disease / Plasma rico em plaquetas no olho seco diabético

ABSTRACT Objective: To evaluate the effectiveness of autologous Plasma- rich platelet (PRP) in symptomatic dry eye of diabetic patients. Design A prospective single group interventional study. Participants: Twelve diabetic patients with refractory dry eye disease. Methods Patients were treated with autologous PRP eye drops four times a day for a month. Dry eye symptoms and its frequency, according to DEWS studies, tear film breakup time, improvement of visual acuity lines, and Schirmer test. Results: All the patients had some symptomatic improvement regarding dryness, itching, burning and redness (p=0.002). Five patients, 41.66% (5/12) had improvement of 1 or more lines of visual acuity in both eyes, 50% and 58.33% had no alteration in right and left eye, respectively (p=0.14). Considering Schirmer test, 66.66% (8/12) had improvement in the test value, 25% (3/12) had no alteration in this test value and 8.33% (1/12) had a reduced value in the test after treatment.(p=0.04). Considering the value of BUT test 58.33% (7/12) had improvement in the test value and 41.66% (5/ 12) had no alteration in this test value (p=0.018). Conclusions PRP is safe and an interesting alternative therapy in symptomatic diabetic dry eye. More clinical trials are required to create specific protocols to this treatment.

RESUMO Objetivo: Avaliar a eficácia do colírio de concentrado de plaquetas (CCP) autólogo no olho seco sintomático de pacientes diabéticos. Projeto Um estudo de intervenção único grupo prospectivo. Participantes Doze pacientes diabéticos com doença do olho seco refratário. Métodos Os pacientes foram tratados com colírio de PRP autólogo quatro vezes por dia durante um mês. Sintomas de olho seco e sua frequência, seguindo os critérios de DEWS, tempo de ruptura do filme lacrimal, a melhoria das linhas de acuidade visual e teste de Schirmer. Resultados Todos os pacientes tiveram alguma melhora dos sintomas de ressecamento, coceira, ardor e vermelhidão (p = 0,002). Destes, 41,66% (5/12) tiveram melhora de uma ou mais linhas de acuidade visual em ambos os olhos; 50% e 58,33% não tinham alteração no olho direito e esquerdo, respectivamente (p = 0,14) . Considerando o teste de Schirmer, 66,66% (8/12) tiveram melhora no valor do teste, 25% (3/12) não apresentaram alteração neste valor de teste e de 8,33% (1/12) tiveram um valor reduzido no teste após tratamento. (p = 0,04). Considerando o valor de teste BUT 58,33% (7/12) apresentaram melhora no valor de teste e 41,66% (12/05) não apresentaram alteração neste valor de teste (p = 0,018). Conclusões O CCP é seguro e uma terapia alternativa interessante no olho seco diabético sintomático. Mais ensaios clínicos são necessários para criar protocolos específicos para este tratamento.

Fatores de risco para aloimunização em pacientes com anemia falciforme / Risk factors for alloimmunization in patients with sickle cell anemia

OBJETIVO: Determinar a imunofenotipagem eritrocitária em doadores de sangue e em pacientes com anemia falciforme (SS) atendidos no Hemocentro de Alagoas e descrever a frequência e os fatores associados à aloimunização eritrocitária. MÉTODOS: Estudo transversal com 102 pacientes SS e 100 doadores de sangue. Realizou-se a fenotipagem eritrocitária, teste de Coombs Direto e Indireto e detecção de anticorpos irregulares por painel de hemácias fenotipadas. Os dados foram comparados por meio do teste de Mann-Whitney, qui-quadrado ou teste exato de Fisher. Para análise dos fatores associados à aloimunização utilizou-se a regressão logística univariada e múltipla. RESULTADOS: Os antígenos mais frequentes entre os pacientes e os doadores foram c, e, M, s, JK(a). Observaram-se diferenças significativas entre as frequências dos fenótipos dos pacientes e dos doadores em relação aos antígenos s, FY(a) e JK(b). Dos 79 pacientes transfundidos, 10 (12,7 por cento) apresentaram Coombs Indireto positivo. Detectaram-se 13 aloanticorpos, sete do sistema Rh, dois do Kell e quatro não identificados. Os fatores associados à aloimunização foram o intervalo de tempo entre a última transfusão e a data do teste e ter recebido mais de dez transfusões de hemácias. Receber mais de dez transfusões representou uma chance 16,39 (IC 95 por cento: 2,23-120,59) vezes maior de ser aloimunizado, em comparação aos que receberam menos que dez. CONCLUSÃO: A prevalência de aloimunização nos pacientes SS foi 12,7 por cento, sendo 70 por cento dos anticorpos encontrados pertencentes a grupos sanguíneos Rh e Kell. Este estudo mostra a importância da fenotipagem eritrocitária em doadores e receptores para diminuir o risco de aloimunização.

OBJECTIVE: To determine erythrocyte phenotyping in blood donors and patients with sickle cell anemia (SS) treated at Hemocentro of Alagoas and describe the frequency and factors associated with erythrocyte alloimmunization. METHODS: Cross-sectional study with 102 SS patients and 100 blood donors. The following tests were performed: erythrocyte phenotyping, Direct and Indirect antiglobulin test, and detection of irregular antibodies by panel of phenotyped red blood cells. Data were compared by Mann-Whitney, qui-square or Fisher’s exact tests. Factors associated with alloimmunization were studied by univariate and multiple logistic regression analysis. RESULTS: The most frequent antigens found in patients and blood donors were: c, e, M, s, JK(a). Significant differences were observed between the frequency of the phenotype of patients and donors in regard to antigens s, FY(a) and JK(b). Of 79 transfused patients, 10 presented positive Indirect Coombs. Thirteen alloantibodies were found, 7 of the Rh system, 2 of Kell and 4 were not identified. Factors associated with alloimmunization were the period of time between the last transfusion and the date of the test and more than 10 red blood cell transfusions. Patients who received more than 10 transfusions were 16.39 (95 percent CI: 2.23-120.59) times more likely to be alloimmunized than patients with fewer transfusions. CONCLUSION: The prevalence of alloimmunization in SS patients was 12.7 percent, with 70 percent of antibodies belonging to the Rh and Kell systems. This study shows the importance of performing erythrocyte phenotyping in blood donors and receptors to decrease the risk of alloimmunization.

Risk factors for alloimmunization in patients with sickle cell anemia.

OBJECTIVE: To determine erythrocyte phenotyping in blood donors and patients with sickle cell anemia (SS) treated at Hemocentro of Alagoas and describe the frequency and factors associated with erythrocyte alloimmunization. METHODS: Cross-sectional study with 102 SS patients and 100 blood donors. The following tests were performed: erythrocyte phenotyping, Direct and Indirect antiglobulin test, and detection of irregular antibodies by panel of phenotyped red blood cells. Data were compared by Mann-Whitney, chi-square or Fisher's exact tests. Factors associated with alloimmunization were studied by univariate and multiple logistic regression analysis. RESULTS: The most frequent antigens found in patients and blood donors were: c, e, M, s, JK(a). Significant differences were observed between the frequency of the phenotype of patients and donors in regard to antigens s, FY(a) and JK(b). Of 79 transfused patients, 10 presented positive Indirect Coombs. Thirteen alloantibodies were found, 7 of the Rh system, 2 of Kell and 4 were not identified. Factors associated with alloimmunization were the period of time between the last transfusion and the date of the test and more than 10 red blood cell transfusions. Patients who received more than 10 transfusions were 16.39 (95% CI: 2.23-120.59) times more likely to be alloimmunized than patients with fewer transfusions. CONCLUSION: The prevalence of alloimmunization in SS patients was 12.7%, with 70% of antibodies belonging to the Rh and Kell systems. This study shows the importance of performing erythrocyte phenotyping in blood donors and receptors to decrease the risk of alloimmunization.