Consensus of the Brazilian association of hematology, hemotherapy and cellular therapy on patient blood management: Anemia tolerance.

Anemia is a pathological condition in which the hemoglobin and red blood cell mass decrease; it is mainly defined by the concentration of hemoglobin in the blood. The World Health Organization guidelines establish specific values to define anemia in different population groups. Early detection of anemia can also be a valuable indicator of underlying medical conditions. Clinical studies have explored the relationship between perioperative anemia and morbidity, highlighting the need for more judicious therapeutic strategies, such as the use of Patient Blood Management, which aims to prevent and treat anemia in a personalized and effective way. Patient Blood Management emerges as a promising approach to dealing with anemia, recognizing that its correction through transfusion always carries risks and that personalized prevention and treatment can offer better outcomes for patients.

Consensus of the Brazilian association of hematology, hemotherapy and cellular therapy on patient blood management: Anemia tolerance mechanisms.

Understanding the physiological concepts of oxygen delivery is essential to discern the mechanisms that influence its increase, reduction or maintenance in the body. This text explores the different mechanisms that help maintain oxygen delivery even in the face of reduced hemoglobin levels. Adequate oxygen delivery ensures tissue and metabolic balance, which is crucial to avoid harmful consequences such as metabolic acidosis and cellular dysoxia. The complex interaction between variables such as cardiac output, hemoglobin and heart rate (HR) plays a fundamental role in maintaining oxygen delivery, allowing the body to temporarily adjust to situations of anemia or high metabolic demand. It is important to emphasize that blood transfusions should not be based on fixed values, but rather on individual metabolic needs. Strategies to reduce myocardial consumption and monitor macro and micro hemodynamics help in making rational decisions. Individualizing treatment and considering factors such as blood viscosity in relation to the benefits of transfusion are increasingly relevant to optimize therapy and minimize risks, especially in complex clinical scenarios, such as neurocritical patients and trauma victims.

Consensus of the Brazilian association of hematology, hemotherapy and cellular therapy on patient blood management: Assessment and management of postoperative anemia.

Postoperative anemia is a complex clinical issue that requires attention due to its ramifications on the patient's recovery and prognosis. Originating from multiple determinants, such as intraoperative blood loss, hemolysis, nutritional deficiencies, systemic inflammation and impact on the bone marrow, postoperative anemia has varied and often challenging presentations. Patients undergoing major surgical procedures, in particular, are susceptible to developing anemia due to the considerable associated blood loss. Accurate diagnosis plays a crucial role in the approach, requiring meticulous hematological analysis, including hemoglobin, hematocrit and reticulocyte count, as well as an in-depth investigation of the underlying causes. An additional challenge arises in the form of the excessive practice of phlebotomy during hospitalization for clinical monitoring. Although it is essential to assess the progression of anemia, frequent removal of blood may contribute to iatrogenic anemia, further delaying recovery and possibly increasing susceptibility to infection.

Consensus of the Brazilian association of hematology, hemotherapy and cellular therapy on patient blood management: Assessment and management of postoperative anemia

Abstract Postoperative anemia is a complex clinical issue that requires attention due to its ramifications on the patient's recovery and prognosis. Originating from multiple determinants, such as intraoperative blood loss, hemolysis, nutritional deficiencies, systemic inflammation and impact on the bone marrow, postoperative anemia has varied and often challenging presentations. Patients undergoing major surgical procedures, in particular, are susceptible to developing anemia due to the considerable associated blood loss. Accurate diagnosis plays a crucial role in the approach, requiring meticulous hematological analysis, including hemoglobin, hematocrit and reticulocyte count, as well as an in-depth investigation of the underlying causes. An additional challenge arises in the form of the excessive practice of phlebotomy during hospitalization for clinical monitoring. Although it is essential to assess the progression of anemia, frequent removal of blood may contribute to iatrogenic anemia, further delaying recovery and possibly increasing susceptibility to infection.

Consensus of the Brazilian association of hematology, hemotherapy and cellular therapy on patient blood management: Anemia tolerance mechanisms

Abstract Understanding the physiological concepts of oxygen delivery is essential to discern the mechanisms that influence its increase, reduction or maintenance in the body. This text explores the different mechanisms that help maintain oxygen delivery even in the face of reduced hemoglobin levels. Adequate oxygen delivery ensures tissue and metabolic balance, which is crucial to avoid harmful consequences such as metabolic acidosis and cellular dysoxia. The complex interaction between variables such as cardiac output, hemoglobin and heart rate (HR) plays a fundamental role in maintaining oxygen delivery, allowing the body to temporarily adjust to situations of anemia or high metabolic demand. It is important to emphasize that blood transfusions should not be based on fixed values, but rather on individual metabolic needs. Strategies to reduce myocardial consumption and monitor macro and micro hemodynamics help in making rational decisions. Individualizing treatment and considering factors such as blood viscosity in relation to the benefits of transfusion are increasingly relevant to optimize therapy and minimize risks, especially in complex clinical scenarios, such as neurocritical patients and trauma victims.

Assessment of the need for routine intraoperative cell salvage in liver transplantation.

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.

Assessment of the need for routine intraoperative cell salvage in liver transplantation

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.

Maternal-fetal alloimmunization: perinatal outcomes in a reference hospital in Northeastern Brazil.

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.

Maternal-fetal alloimmunization: perinatal outcomes in a reference hospital in Northeastern Brazil

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.

The use of social media as a tool for patient blood management and transfusion medicine education.

BACKGROUND AND OBJECTIVES: Transfusion is one of the most performed medical procedures. Wrong indications are common and are probably related to the scarcity of transfusion teaching during medical education. The development of a new way to improve transfusion education is paramount. Social media has the potential to reach larger audiences for rapid communication of medical content. The use of social media for transfusion education in Brazil has not been published. The aim of this article is to describe a new tool to improve transfusion learning. MATERIALS AND METHODS: Evidence-based cards were created. Initially, these cards were sent by WhatsApp. Later, Instagram and Facebook pages were created. EducaSangue, as this e-learning project was called, is a tool for the spreading of transfusion knowledge that permits the exchange of experiences. RESULTS: By April 2021, Facebook and Instagram pages had 8300 and 5100 followers, respectively. Cards about single red blood cell (RBC) unit, alternatives to transfusion, transfusion reactions and pre-transfusion tests were published. Doctors and other health professionals follow EducaSangue. RBC transfusions reduced in Ceara and single-unit RBC increased by 28%, although not statistically significant. In Brazil, the minority of medical schools have transfusion as a discipline. The scarcity of transfusion education is related to the poorer care of the patient. Technological innovation has been used for educational changes and is an alternative to formal education. CONCLUSION: Social media is an interesting tool to provide quality to medical services, since they can reach a broader public, especially where personal contact is difficult.

Subjective donor deferral as a tool for increased blood transfusion safety: A cross-sectional observational study.

OBJECTIVES: This study aims at evaluating whether subjective donor deferral (SDD) has the potential for increasing blood transfusion safety. BACKGROUND: Appropriate donor selection via clinical and serologic screening is necessary to prevent transfusion-transmissible infections (TTIs). One additional strategy adopted by some Brazilian blood transfusion centers (BTCs) is the rejection of a donation by the pre-donation interviewer based on subjective factors. METHODS/MATERIALS: We conducted a STROBE-guided cross-sectional study including 105 005 prospective donors who presented to our BTC between 1 January 2013, and 31 December 2015. Donors were evaluated for age, gender, education level, donation type and history, confidential unit exclusion, SDD, and results of serologic screening for TTIs. RESULTS: Even after controlling for potential confounding variables, subjectively deferred donors were more likely to have at least one reactive serology in the standard screening (OR: 2.80; 95% CI: 2.13-3.69; P < .001). They also had a higher risk for testing positive for syphilis (OR: 4.47; 95% CI: 3.05-6.55; P < .001), hepatitis B (OR: 5.69; 95% CI: 2.48-13.08; P < .001), and HIV (OR: 6.14; 95% CI: 3.22-11.69; P < .001). CONCLUSIONS: Routine implementation of SDD in donor selection may be an effective additional measure to avoid TTIs, highlighting the importance of interviewer experience, perspicacity, and face-to-face contact with donors for blood safety assurance.

Prospective evaluation of immune haemolysis in liver transplantation.

BACKGROUND: Immune haemolysis in liver transplant (LT) can occur due to autoantibodies and alloantibodies. The aim of this study was to evaluate the prevalence and risk factors for immune haemolysis in LT. METHODS: A total of 175 consecutive patients were included. Multiorgan recipients were excluded. Samples, from before LT, seven consecutive days and weekly for 4 weeks, were evaluated for haemolysis and immunohaematological tests. SPSS 24 was used for statistical analysis. RESULTS: Nine patients (5·1%) presented positive antibody screen (AS) before LT, (2·3% clinically significant), more frequent in RhD-negative (P = 0·017). Positive DAT occurred in 53 (30·3%) and was related to high MELD score (P = 0·048), HCV (P = 0·005) and furosemide use (P = 0·001). Positive AS after LT occurred in 22 patients (12·5%), with nine (5·7%) clinically significant antibodies. Positive AS occurred more frequently in RhD negative (P = 0·021) and in those transfused (P = 0·022). Post-transplant positive DAT was associated with piperacillin-tazobactam use (P = 0·021) and minor ABO incompatibility (P = 0·0038). Five patients presented passenger lymphocyte syndrome (PLS), all received liver-graft O, four presented haemolysis, and three were transfused due to PLS. CONCLUSION: Auto- and alloantibodies against red blood cell antigens are frequent in LT, but the frequency of immune haemolysis was only 2·8%. The only risk factor for PLS was minor ABO mismatch.

A blockage monoclonal antibody protocol as an alternative strategy to avoid anti-CD38 interference in immunohematological testing.

BACKGROUND: As CD38 is expressed on red blood cells (RBCs), the plasma of patients on daratumumab (DARA) reacts with the panel cells of pretransfusion tests, masking underlying alloantibodies. The treatment of RBCs with dithiothreitol (DTT) is the most disseminated method to overcome DARA effect on immunohematological tests, but it hampers the identification of potentially harmful antibodies. Our goal was to validate a new strategy, the blockage monoclonal antibody protocol (BMAP), to mitigate the DARA interference on RBCs using anti-CD38 and antihuman globulin. METHODS: Samples of patients receiving DARA were included in the study. Sera were tested using both DTT- and BMAP-treated RBCs, which comprised three steps: 1) titration of monoclonal anti-CD38, 2) treatment of RBCs obtained from donors with anti-CD38, and 3) blockage of anti-CD38-adsorbed RBCs with antihuman globulin. RESULTS: Twenty patients were included in the study. Donor RBCs were treated with anti-CD38 and successfully blocked with antihuman globulin. In 19 patients, DARA-mediated agglutination was eliminated using both DTT- and BMAP-treated RBCs. In one patient, agglutination persisted when tested against the BMAP-treated RBCs, and alloantibodies were identified. Patient samples were mixed with commercial anti-D, -C, -e, -K, -Jka, -Kpb and tested against antigen-positive BMAP-treated RBCs, resulting in detection of these antibodies. CONCLUSION: This study validated a new strategy to minimize the interference of DARA on immunohematological tests. The protocol preserves the integrity of RBC antigens, permitting the detection of antibodies from all blood group systems. The BMAP has potential use in other situations where specific antibodies may interfere with pretransfusion screening.

Renal involvement in paroxysmal nocturnal haemoglobinuria: a brief review of the literature.

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.

Renal involvement in paroxysmal nocturnal haemoglobinuria: a brief review of the literature

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.

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.

Successful hematopoietic stem cell mobilization with vinorelbine and filgrastim in germ cell tumor.

Germ cell tumor (GCT) is the most frequent cancer in young men and is highly curable. Almost 80 % of patients with the disease in an advanced stage achieve a reliable response to cisplatin combination chemotherapy. For relapsing or refractory disease, autologous hematopoietic stem cell transplantation (HSCT) is an effective therapy. The two most used mobilization strategies for HSC collection are filgrastim alone or filgrastim after chemotherapy (chemomobilization). HSC collection with filgrastim mobilization can be difficult, especially in highly treated patients. While the addition of chemotherapy improves mobilization and reduces the number of apheresis sessions, it can increase morbidity rate as well. We describe a case of a 45-year-old male with classical seminoma who was submitted to orchiectomy. Two months after, he presented progression of the tumor. He received four cycles of cisplatin, etoposide and bleomycin, with residual retroperitoneal mass and cervical lymphadenopathy. Further, he was submitted to three more cycles of cisplatin, ifosfamide and paclitaxel. Thereupon, he showed partial response. At that moment, autologous HSC transplantation was considered. In the first mobilization, filgrastim alone was used without success in harvesting. The second mobilization consisted of vinorelbine at day 1 (35 mg/m2) and filgrastim (16 µg/kg) started at day 5. The peak of CD34+ cells in peripheral blood was 32.6 × 106 cells/L on day 8, with 4.73 × 106 cells/kg CD34+ collected on days 8 and 9. The benefits of this scheme include: (a) outpatient administration, (b) fewer doses of filgrastim, (c) minimal risk of febrile neutropenia and (d) reliable prediction of collection day. For these reasons, we conclude that vinorelbine chemomobilization is a great option for GCT, particularly in patients with high risk of mobilization failure. Furthermore, it requires less resource usage, hospitalizations and transfusions than conventional chemomobilization.

Hydroxyurea increases plasma concentrations of microparticles and reduces coagulation activation and fibrinolysis in patients with sickle cell anemia.

Microparticles (MPs) are present in healthy subjects and their concentration increases in patients at high risk of thrombosis. We evaluated 10 patients with sickle cell anemia (SCA) treated with hydroxyurea (HU) and 13 SCA patients without this treatment. MP concentrations were determined by flow cytometry. Coagulation was evaluated using the thrombin-antithrombin complex (TAT) and D-dimers. Total MP concentrations were increased in the HU-treated group (265 × 10(6)/ml vs. 67.45 × 10(6)/ml; p = 0.0026), as well as MPs derived from RBC (67.83 × 10(6)/ml vs. 26.31 × 10(6)/ml; p = 0.05), monocytes (51.31 × 10(6)/ml vs. 9.03 × 10(6)/ml; p = 0.0084), monocytes with tissue factor (TF) expression (2.27 × 10(6)/ml vs. 0.27 × 10(6)/ml; p = 0.0058), endothelium (49.42 × 10(6)/ml vs. 7.23 × 10(6)/ml; p = 0.007) and endothelium with TF (1.42 × 10(6)/ml vs. 0.26 × 10(6)/ml; p = 0.0043). Furthermore, the concentrations of TAT (7.56 vs. 10.98 µg/l; p = 0.014) and D-dimers (0.65 vs. 1.29 µg/ml; p = 0.007) were reduced with HU. The MP elevation may suggest a direct cytotoxic effect of HU. Another explanation is a cell surface increase secondary to a megaloblastic process, resulting in increased vesicle release. In our opinion, the known benefits of HU on SCA patients, along with the reduction in coagulation activation, surpass its potential detrimental effect on MPs. Future studies should elucidate the role of MPs and demonstrate their significance in different contexts.

Sickle cell disease and pregnancy: analysis of 34 patients followed at the Regional Blood Center of Ribeirão Preto, Brazil.

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±643.8g. 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.