Role of complement in alloimmunization and hyperhemolysis
Purpose of review The purpose of this review is to summarize the role of complement in regulating the removal of a target alloantigen following an incompatible red blood cell (RBC) transfusion, the formation of alloantibodies following RBC alloantigen exposure, and the development of hyperhemolysis in patients with sickle cell disease (SCD). Recent findings Recent studies demonstrate that complement can accelerate alloantibody-mediated removal of target alloantigens from the RBC surface following incompatible transfusion. Complement also influences alloantigen availability during developing alloimmune responses and serves as a unique mediator of CD4 T-cell-independent alloantibody formation following RBC alloantigen exposure. Finally, alternative complement pathway activation appears to play a key role in the development of acute hemolytic episodes in patients with SCD, providing a potential druggable target to prevent acute complications in patients with this disease. Summary Recent studies suggest that complement can regulate a wide variety of processes germane to hematology, from transfusion complications to baseline hemolysis in patients with SCD. As the role of complement in various disease processes becomes more fully understood, the ability to leverage recently developed complement modulating drugs will only continue to enhance providers’ ability to favorably intervene in many hematological diseases.
We describe the most highly recommended generic and disease-specific PRO tools in SCD and discuss the challenges of incorporating them in clinical practice. EXPERT OPINION: PRO measures are essential to incorporate into SCD clinical trials either as primary or secondary outcomes. The use of PRO measures in SCD facilitates a patient-centered approach, which is likely to lead to improved outcomes. Significant challenges remain in adapting PRO tools to routine clinical use and in developing countries. PMID: 33034214 [PubMed - as supplied by publisher]
Publication date: Available online 9 October 2020Source: Blood Cells, Molecules, and DiseasesAuthor(s): Melissa Azul, Surbhi Shah, Sarah Williams, Gregory M. Vercellotti, Alexander A. Boucher
Publication date: Available online 8 October 2020Source: Hematology/Oncology and Stem Cell TherapyAuthor(s): M.M. Sulaiman, A.I. Ladu, A.M. Abba, A.A. Bukar
Condition: Sickle Cell Anemia Intervention: Drug: Voxelotor Sponsors: Elizabeth Yang; Mednax Center for Research, Education, Quality and Safety; University of California, San Francisco; Global Blood Therapeutics Recruiting
AbstractSickle cell disease (SCD) is a genetic disorder predominantly affecting people of African descent and is associated with significant morbidity and mortality. To improve SCD outcomes, the National Heart Lung and Blood Institute funded eight centers to participate in the SCD Implementation Consortium. Sites were required to each recruit 300 individuals with SCD, over 20 months. We aim to describe recruitment strategies and challenges encountered. Participants aged 15–45 years with confirmed diagnosis of SCD were eligible. Descriptive statistics were used to analyze the effectiveness of each recruitme...
ConclusionThe prevalence of RLS among this sample of adult patients with sickle cell anemia was much higher than that previously reported for the general Turkish population. The presence of RLS was an important cause of sleep disturbance in these adult patients with SCA.
CONCLUSION: The prevalence of RLS among this sample of adult patients with sickle cell anemia was much higher than that previously reported for the general Turkish population. The presence of RLS was an important cause of sleep disturbance in these adult patients with SCA. PMID: 33029692 [PubMed - as supplied by publisher]
Conclusion: The risk of pregnancy in patients with homozygous sickle cell anemia remains high, on both the maternal and fetal sides. PMID: 33014460 [PubMed]
Abstract Total pancreatectomy with islet autotransplantation is a complex surgical approach for acute recurrent or chronic pancreatitis that frequently triggers extreme thrombocytosis (platelets ≥ 1000 × 109 /L). Thrombocytosis can be prothrombotic, so cytoreductive hydroxyurea is often initiated after this surgery; however, optimal dosing strategy and efficacy are unknown. This prospective pilot study characterized the pharmacokinetics of hydroxyurea after this procedure in children. It also compared them with previously published pediatric parameters in sickle cell anemia (SCA), the disease in which ped...
(University of Illinois at Chicago) Researchers at the University of Illinois Chicago will lead a $7.1 million, five-year national study to determine the effectiveness of acupuncture and guided relaxation for people with chronic pain from sickle cell disease. The National Institutes of Health grant is part of its Helping to End Addiction Long-term Initiative. The initiative aims to improve prevention and treatment strategies for opioid misuse and addiction and to enhance pain management.