ABSTRACT Purpose: This study aimed to assess the necessity of routine intraoperative cell salvage in liver transplantations. Methods: A total of 327 liver transplants performed between 2014 and 2016 was included in the analysis. Patient data, including pre-transplant examinations, intraoperative red blood cell transfusions, and procedural information, were collected. Results: The median age of the patients was 54 years old, with 67% (219) being male. The most prevalent ABO blood type was O, accounting for 48% (155) of cases. The leading causes of liver disease were hepatitis C (113 cases, 34.6%) and alcohol-related liver disease (97 cases, 29.7%). Out of the 327 liver transplants, allogeneic red blood cell transfusions were administered in 110 cases (34%) with a median of two units of red blood cells per case. Cell salvage was employed in 237 transplants (73%), and successful blood recovery was achieved in 221 cases (93%). Among the group that recovered more than 200 mL of blood, the median volume of recovered blood was 417 mL, with no transfusion of allogeneic blood required. A total of 90 transplants was performed without utilizing cell salvage, and, among these cases, 19 required blood transfusions, with a median of zero units transfused. Conclusions: This study suggests that routine cell salvage is unnecessary for all liver transplantations. The most suitable indication for its use is in patients presenting with portal vein thrombosis and abnormal creatinine levels.

SUMMARY OBJECTIVE: To assess the prevalence of maternal alloantibodies in pregnant women at a maternity hospital in northeastern Brazil and describe their perinatal outcomes. METHODS: A retrospective cohort study reviewed maternal and newborn medical records between January 2017 and October 2018 to assess for the presence of maternal alloantibodies. RESULTS: The following maternal alloantibodies were found in the 41 cases surveyed: anti-D, 28 cases (45%); anti-C, 7 cases (11%); anti-c, 1 case (1.6%); anti-E, 4 cases (6.4%); anti-Cw, 1 case (1.6%); anti-K, 2 cases (3.2%); anti-Jka, 1 case (1.6%); anti-M, 3 cases (4.8%); anti-Fya, 2 cases (3.2%); anti-Fyb, 1 case (1.6%); anti-Lea, 5 cases (8%); anti-Leb, 3 cases (4.8%); and anti-Dia, 4 cases (6.4%). Anti-D antibodies were the most frequent cause of erythrocyte alloimmunization (80%). Fetal anemia was observed in four pregnancies based on the peak systolic velocity of the middle cerebral artery. In one case, the mother showed anti-M, and anti-Lea alloimmunization, but the direct antiglobulin test results for the newborn were negative, and no unfavorable neonatal outcomes were observed. In one case of a mother with anti-C and anti-D alloimmunization, the neonate showed anti-D antibodies only in the serological panel and required phototherapy. Neonates with plasma antibodies and jaundice requiring phototherapy only had a serological panel with anti-D, anti-C, anti-c, and anti-E antibodies. Intervention was required for 2.5% of pregnant women with positive antibody screens and 81% of newborns with positive direct antiglobulin test results. CONCLUSION: Despite being a rare condition, maternal alloimmunization by irregular antibodies can result in high perinatal morbidity and mortality.

RESUMO INTRODUÇÃO: A hemoglobinúria paroxística noturna (HPN) é uma doença genética adquirida, caracterizada por hemólise mediada pelo sistema complemento, eventos trombóticos e citopenias variáveis. Envolvimento renal pode ocorrer, contribuindo com morbidade significativa nesses pacientes. OBJETIVO: Realização de revisão de literatura sobre o envolvimento renal na HPN. MÉTODOS: Pesquisa on-line na base de dados Medline, com compilação e análise dos 26 estudos encontrados de maior relevância. RESULTADOS: A HPN pode se apresentar com insuficiência renal aguda induzida por hemólise maciça, que geralmente tem apresentação grave. Em quadros crônicos, declínio insidioso da função renal pode ocorrer por depósitos tubulares de hemossiderina, microinfartos renais e fibrose intersticial. Apesar de o transplante de células-tronco hematopoiéticas permanecer como a única terapia curativa para a HPN, a droga Eculizumab é capaz de melhorar a função renal, entre outros desfechos, por meio da inibição de C5 e a subsequente ativação da cascata do complemento, que culminaria com a formação do complexo de ataque à membrana. CONCLUSÃO: Há poucas informações na literatura no que concerne ao envolvimento renal na HPN, apesar de ser possível estabelecer que os mecanismos fisiopatológicos das lesões agudas e crônicas são distintos. Apesar de não ser uma terapia curativa, Eculizumab é capaz de amenizar o comprometimento renal nesses pacientes.

SUMMARY INTRODUCTION: Paroxysmal Nocturnal Haemoglobinuria (PNH) is an acquired genetic disorder characterized by complement-mediated haemolysis, thrombosis and variable cytopenias. Renal involvement may occur and causes significant morbidity to these patients. OBJECTIVE: To review the literature about pathophysiology and provide recommendations on diagnosis and management of renal involvement in PNH. METHODS: Online research in the Medline database with compilation of the most relevant 26 studies found. RESULTS: PNH may present with acute kidney injury caused by massive haemolysis, which is usually very severe. In the chronic setting, PNH may develop insidious decline in renal function caused by tubular deposits of hemosiderin, renal micro-infarcts and interstitial fibrosis. Although hematopoietic stem cell transplantation remains the only curative treatment for PNH, the drug Eculizumab, a humanized anti-C5 monoclonal antibody is capable of improving renal function, among other outcomes, by inhibiting C5 cleavage with the subsequent inhibition of the terminal complement pathway which would ultimately give rise to the assembly of the membrane attack complex. CONCLUSION: There is a lack of information in literature regarding renal involvement in PNH, albeit it is possible to state that the pathophysiological mechanisms of acute and chronic impairment differ. Despite not being a curative therapy, Eculizumab is able to ease kidney lesions in these patients.

Objective: The objective of this study was to verify the evolution of pregnancies in sickle cell patients followed at one institution over a period of 12 years (January 2000 to June 2012). Methods: The study evaluated 34 pregnant women with sickle cell disease with a mean age of 23.9 ± 5.3 years. The incidence of obstetric complications, non-obstetric complications linked to sickle cell disease and complications in the newborn were analyzed. Results: A total of 26% of the cases reported previous miscarriages, 20% had preterm labor, 10% had pre-eclampsia, and 5% had gestational diabetes. Forty-one percent of the deliveries were cesarean sections and 29% of patients required blood transfusions. In respect to sickle cell disease, 62% of patients had vaso-occlusive crises, 29% had acute chest syndrome, 23% had urinary tract infection, 15% had impaired cardiac function and 6% developed pulmonary hypertension. Only one patient died in the postnatal period due to acute chest syndrome. The mean gestational age was 37.8 ± 2.63 weeks, and mean newborn weight was 2.809 ± 643.8 g. There were seven fetal losses, including three stillbirths and four miscarriages. The impact of transfusion therapy on the incidence of maternal–fetal complications during pregnancy was evaluated. Conclusions: Pregnancy in sickle cell patients is still associated with complications. Although no statistical difference was observed between transfused and non-transfused women, there were no deaths (fetal or maternal) in transfused patients whereas one maternal death and three stillbirths occurred in non-transfused women. A larger study of sickle cell pregnant women will be necessary to elucidate the actual role of transfusion during pregnancy in sickle cell disease.

HIV patients infected through injected drug use have poorer prognosis than other groups. We evaluated the hematological alterations and rates of co-infections in injected drug use patients with AIDS. Injected drug use patients were younger, predominantly of male gender, and presented lower CD4, total lymphocyte, and platelet counts, but not neutrophil count, than control group. Injected drug use patients had a higher rate of hepatitis C and mycobacteria infection. Furthermore, all injected drug use patients with hemoglobin <10.0 g dL-1 and lymphocyte <1000 µL-1 had CD4 count lower than 100 µL-1. In conclusion, HIV-infected injected drug use patients constitute a special group of patients, and hemoglobin concentration and lymphocyte count can be used as surrogate markers for disease severity.

CONTEXTO E OBJETIVO A doença falciforme (SCD) é o distúrbio genético mais comum entre afrodes-cendentes, afetando aproximadamente 3.500 recém-nascidos a cada ano no Brasil. A hidroxiureia (HU) é a única droga efetiva para o tratamento dos pacientes com SCD, reduzindo a morbidade e a mortalidade da doença. O objetivo do estudo foi analisar os efeitos da HU em pacientes com SCD em nossa instituição. TIPO DE ESTUDO E LOCAL Estudo retrospectivo realizado em um centro de anemia falciforme em Ribeirão Preto, São Paulo, Brasil. MÉTODOS Nós analisamos os dados clínicos e laboratoriais de 37 pacientes. Os parâmetros hematológicos e eventos clínicos que ocorreram no ano anterior e durante o primeiro ano de tratamento com HU foram analisados. A dose média de HU foi 24.5 ± 5.5 mg/kg/dia. RESULTADOS Houve aumento em três parâmetros estudados: hemoglobina (8,3 g/dl para 9,0 g/dl, P = 0,0003), hemoglobina fetal (HbF) (2,6% para 19,8%, P < 0,0001) e volume corpuscular médio (VCM) (89 para 105 fl, P = 0,001); e redução do número de leucócitos (10.050/µl para 5.700/µl, P < 0,0001), neutrófilos (6.200/µl para 3.400/µl, P = 0,001), plaquetas (459.000/µl para 373.000/µl, P = 0,0002), crises dolorosas (1,86 para 0,81, P = 0,0014), síndrome torácica aguda (0,35 para 0,08, P = 0,0045), infecções (1,03 para 0,5, P = 0,047), hospitalizações (1,63 para 0,53, P = 0,0013) e número de transfusões (1,23 para 0,1, P = 0,0051). CONCLUSÃO Os pacientes apresentaram melhora clínica e hematológica, com aumento da HbF e redução da taxa de infecção, dado este não explorado na maioria dos estudos clínicos já publicados.

CONTEXT AND OBJECTIVES Sickle cell disease (SCD) is the most common genetic disorder among people of African descent, affecting approximately 3,500 newborns each year in Brazil. Hydroxyurea (HU) is the only effective drug to treating patients with SCD, thereby reducing morbidity and mortality. The objective was to analyze the effects of HU on SCD patients at our institution. DESIGN AND SETTING Retrospective study conducted at a sickle cell centre in Ribeirão Preto, São Paulo, Brazil. METHODS We analyzed clinical and laboratory data on 37 patients. The hematological parameters and clinical events that occurred during the year before and the first year of treatment with HU were analyzed. The mean dose of HU was 24.5 ± 5.5 mg/kg/day. RESULTS There were rises in three parameters: hemoglobin (8.3 g/dl to 9.0 g/dl, P = 0.0003), fetal hemoglobin (HbF) (2.6% to 19.8%, P < 0.0001) and mean cell volume MCV (89 to 105 fl, P = 0.001); and reductions in the numbers of leukocytes (10,050/µl to 5,700/µl, P < 0.0001), neutrophils (6,200/µl to 3,400/µl, P = 0.001), platelets (459,000/µl to 373,000/µl, P = 0.0002), painful crises (1.86 to 0.81, P = 0.0014), acute chest syndromes (0.35 to 0.08, P = 0.0045), infections (1.03 to 0.5, P = 0.047), hospitalizations (1.63 to 0.53, P = 0.0013) and transfusions (1.23 to 0.1, P = 0.0051). CONCLUSION The patients presented clinical and hematological improvements, with an increase in HbF and a reduction in the infection rate, which had not been addressed in most previous studies.

OBJECTIVE: Sickle cell anemia and the interaction S/Beta thalassemia differ in hematological values due to microcytosis and hypochromia caused by the thalassemic mutation. The clinical benefit of long-term hydroxyurea treatment is undeniable in sickle cell disease with monitoring of the biological action of the drug being by the complete blood count. The objective of this work is to compare changes in some of the erythrocytic indexes between S/Beta thalassemia and sickle cell anemia patients on long-term hydroxyurea treatment. METHODS: The values of erythrocyte indexes (mean corpuscular volume and mean corpuscular hemoglobin) were compared in a retrospective study of two groups of patients (Sickle cell anemia and S/Beta thalassemia) on hydroxyurea treatment over a mean of six years. RESULTS: The quantitative values of the two parameters differed between the groups. Increases in mean corpuscular volume and reductions in mean corpuscular hemoglobin delay longer in S/Beta thalassemia patients (p-value = 0.018). CONCLUSION: Hematological changes are some of the beneficial effects of hydroxyurea in sickle cell disease as cellular hydration increases and the hemoglobin S concentration is reduced. The complete blood count is the best test to monitor changes, but the interpretation of the results in S/Beta thalassemia should be different.

CONTEXTO: Rabdomiólise é uma condição clínica severa e ameaçadora à vida, em que ocorre destruição de músculo esquelético. Insuficiência renal aguda por rabdomiólise já foi bem descrita, sendo os principais mecanismos fisiopatológicos vasoconstrição renal, formação de depósitos intratubulares e toxicidade direta da mioglobina. TIPO DE ESTUDO: Relato de caso e revisão da literatura. OBJETIVO: Relatar um caso de insuficiência renal aguda (IRA) induzida por rabdomiólise devida a exercícios físicos intensos e uso abusivo de álcool e descrever os principais mecanismos fisiopatológicos deste tipo de IRA. RELATO DE CASO: Homem de 39 anos foi admitido na emergência com edema e síndrome compartimental em membros inferiores. Ele estava oligúrico, e os níveis séricos de creatinina e uréia eram de 8,1mg/dl e 195mg/dl, respectivamente. O diagnóstico de rabdomiólise foi obtido através dos dados da história clínica e dos exames laboratoriais (creatinoquinase de 26320 UI/l). O tratamento inicial consistiu em reposição volêmica e diurese forçada. Tratamento específico para síndrome compartimental, como fasciotomia, não foi realizado para que se evitasse a ocorrência de infecções. Recuperação parcial da função renal foi observada após 10 sessões de hemodiálise. Recuperação completa ocorreu após dois meses de acompanhamento.

CONTEXT: Rhabdomyolysis is a severe and life-threatening condition in which skeletal muscle is damaged. Acute renal failure due to rhabdomyolysis has been widely described and its main pathophysiological mechanisms are renal vasoconstriction, intraluminal cast formation and direct myoglobin toxicity. OBJECTIVE: To report on a case of acute renal failure (ARF) induced by rhabdomyolysis due to strenuous exercise and alcohol abuse and to describe the pathophysiology of this type of ARF. CASE REPORT: A 39-year-old man arrived at the hospital emergency service with swollen legs and lower extremity compartment syndrome. He was oliguric and had serum creatinine and urea levels of 8.1 mg/dl and 195 mg/dl, respectively. The diagnosis of rhabdomyolysis was made through clinical and laboratory findings (creatine kinase activity of 26320 IU/l). The initial treatment consisted of fluid replacement and forced diuresis. The specific treatment for compartment syndrome, such as fasciotomy, was avoided in order to prevent infection. Partial recovery of renal function was recorded, after ten hemodialysis sessions. Complete recovery was observed after two months of follow-up.