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Key Clinical Message This manuscript describes the case of a patient with sickle cell anemia who died of fulminant hepatitis after therapy with the iron chelator Deferasirox. The patient was homozygous for the −1774delG polymorphism in the Abcc2 gene, which raises the concern about the use of hepatotoxic drugs in this specific context. [ABSTRACT FROM AUTHOR]

Background: Pregnancy represents a challenge for women with sickle cell disease (SCD), with higher rates of both maternal and fetal complications. The aim of this study was to evaluate the impact of prophylactic transfusion support administered specifically to pregnant women with sickle hemoglobin C disease.Materials and Methods: Patients were divided into two groups according to the type of transfusion support received: 10 women received prophylactic erythrocytapheresis or manual exchange transfusion at 28 weeks of gestation, and 14 received transfusions only on demand, due to acute complications, or received no transfusions at all.Results: Our results indicated higher frequencies of SCD-related complications in the group that did not receive prophylactic transfusion support (35.7% vs. only 10% in the erythrocytapheresis group). Furthermore, these complications were more severe in this group and included all cases of acute chest syndrome. A significant difference was observed concerning gestational age at birth (38.7 weeks in the transfusion group vs. 34.4 weeks, p = 0.037), with a higher frequency of preterm births in the nontransfused group (69.23% vs. 30% in the transfusion group).Conclusion: We demonstrated a clear reduction of unfavorable outcomes in patients receiving prophylactic transfusions, probably reflecting better maternal and fetal conditions, which corroborated to the more satisfactory indices of vitality, observed in newborns. Considering that erythrocytapheresis or manual exchange transfusions both represent feasible and safe procedures, they could represent important tools for the optimal management of these patients. [ABSTRACT FROM AUTHOR]

Objectives: APAF-1 is a central component of the intrinsic pathway of apoptosis, where APAF-1 dysregulation results in the development of diverse human neoplasms. The aim of this study was to characterize the mRNA expression levels of APAF-1 transcripts in low-risk and high-risk MDS and to elucidate whether the expression levels of APAF-1 transcripts are modulated with increased apoptosis in CD34+ MDS cells undergoing erythroid differentiation. Methods: APAF-1 ( ) expression was verified, by quantitative RT-PCR, in bone marrow aspirates from 33 patients with myelodysplastic syndromes (MDS), at the time of diagnosis, and in erythroid differentiation cultures from CD34+ from normal donors and patients with MDS. Results: APAF-1 expression was significantly higher in low-risk, compared to high-risk MDS, according to IPSS ( P < 0.0001), FAB ( P = 0.0265), and cytogenetic risk ( P = 0.0134). Low-risk MDS-derived differentiated erythroid cells demonstrated an increased expression of APAF-1, compared with normal cells, accompanied by an augmented rate of apoptosis. Conclusions: Increased expression of APAF-1 in low-risk disease and its positive correlation with the apoptotic rate observed during the erythroblast differentiation of low-risk MDS cells may indicate that the modulation of APAF-1, at the transcriptional level, participates in the pathophysiology of MDS. [ABSTRACT FROM AUTHOR]

Background and Objectives: This report describes the implementation of the first pre‐hospital transfusion service in Latin America, with red blood cell concentrates available for immediate use in mobile land units. Materials and Methods: A partnership was established between the Regional Mobile Emergency Service and the Regional Blood Bank, with further development of protocols for the storage, transportation and transfusion of red blood cell concentrates in an ambulance, with the acquisition of special thermal boxes, a heater for blood components and digital thermometers. Results: The Service was developed from May 2019 and implemented in September 2020, in a mobile intensive care unit serving 11 cities, reaching a population of approximately 480 000 inhabitants. Two red blood cell concentrates, type 'O' RhD negative, are available, stored in a special sealed thermal box and with continuous temperature monitoring. A specific transfusion protocol was developed, in accordance with the institutional protocols of the Hospital and its Blood Bank. Conclusion: This is the first description of a pre‐hospital transfusion service implemented in Latin America, with the potential to reduce the number of deaths from civil trauma and which can be extrapolated to other institutions or countries in similar conditions. [ABSTRACT FROM AUTHOR]