Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disease with abnormal hematopoietic stem cells that causes intravascular hemolytic anemia, thrombosis, and peripheral blood cytopenia. It has a chronic progressive course and can be fatal in severe cases if not treated aggressively. Complement inhibitors are the first-line recommended treatment for hemolysis-related symptoms of PNH. With the rapid development of new complement inhibitors, it is critical to quickly screen and confirm the diagnosis, identify patients with complement inhibitor indications, and monitor breakthrough hemolysis and extravascular hemolysis during complement inhibitor therapy. Drawing on the most recent guidelines, works of literature, and meta-reviews from around the world, as well as combining with experience from the experts, this consensus focused on PNH screening principles, the significance of PNH cloning detection, and post-treatment monitoring of terminal complement inhibitors, which may contribute to a better understanding of diagnosis and treatment monitoring in the era of complement inhibitors.
阵发性睡眠性血红蛋白尿症（paroxysmal nocturnal hemoglobinuria，PNH）是一种罕见的造血干细胞异常克隆性疾病，以血管内溶血性贫血、血栓形成和外周血细胞减少为主要表现，呈慢性进展性病程，严重者可危及生命。补体抑制剂是治疗PNH溶血相关症状的一线推荐药物。随着补体抑制剂领域的快速发展，加强对PNH的筛查、快速诊断，判断需要用补体抑制剂治疗的患者，在补体抑制剂治疗过程中监测突破性溶血、血管外溶血等，对患者的生存、生活质量改善有着重要意义。为促进PNH临床诊疗的规范，本共识参考国内外最新指南和文献，荟萃国内外最新研究成果，并结合专家团队经验，聚焦PNH筛查原则、PNH克隆检测意义、末端C5补体抑制剂治疗后监测等问题，旨在为PNH的筛查、诊断和补体抑制剂时代的治疗监测提供参考意见。.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is associated with development of acute hemolytic anemia in the setting of oxidative stress, which can be caused by medication exposure. Regulatory agencies worldwide warn against the use of certain medications in persons with G6PD deficiency, but in many cases, this information is conflicting, and the clinical evidence is sparse. This guideline provides information on using G6PD genotype as part of the diagnosis of G6PD deficiency and classifies medications that have been previously implicated as unsafe in individuals with G6PD deficiency by one or more sources. We classify these medications as high, medium, or low to no risk based on a systematic review of the published evidence of the gene-drug associations and regulatory warnings. In patients with G6PD deficiency, high-risk medications should be avoided, medium-risk medications should be used with caution, and low-to-no risk medications can be used with standard precautions, without regard to G6PD phenotype. This new document replaces the prior Clinical Pharmacogenetics Implementation Consortium guideline for rasburicase therapy in the context of G6PD genotype (updates at: www.cpicpgx.org).

OBJECTIVE: To systematically review available evidence to develop guidelines for diagnosis and treatment of transfusion-associated reactions in dogs and cats.
METHODS: Standardized and systemic evaluation of the literature (identified through Medline via PubMed and Google Scholar searches) was carried out for identified transfusion reaction types in dogs and cats. The available evidence was evaluated using PICO (Population, Intervention, Comparison, Outcome) questions generated for each reaction type. The evidence was categorized by level of evidence (LOE) and quality (Good, Fair, or Poor). Guidelines, diagnostic, and treatment algorithms were generated based on the evaluation of the evidence. Consensus on the final guidelines was achieved through Delphi-style surveys. Draft recommendations were disseminated through veterinary specialty listservs for review and comments, which were evaluated and integrated prior to final publication.
RESULTS: Medline via PubMed and Google Scholar databases were searched. There were 14 Population Intervention Comparison Outcome questions identified and corresponding worksheets were developed focusing on the diagnosis and treatment of transfusion-associated reactions in dogs and cats. Fourteen guidelines and four algorithms were developed with a high degree of consensus.
CONCLUSIONS: This systematic evidence evaluation process yielded recommended diagnostic and treatment algorithms for use in practice. However, significant knowledge gaps were identified, demonstrating the need for additional research in veterinary transfusion medicine.

BACKGROUND: The consistently increasing reports of bacterial resistance and the reemergence of bacterial epidemics have inspired the health and scientific community to discover new molecules with antibacterial potential continuously. Frog-skin secretions constitute bioactive compounds essential for finding new biopharmaceuticals. The exact antibacterial characterization of dermaseptin related peptides derived from Agalychnis annae, is limited. The resemblance in their conserved and functionally linked genomes indicates an unprecedented opportunity to obtain novel bioactive compounds.
OBJECTIVE: In this study, we derived a novel peptide sequence and determined its antibacterial potentials.
METHODS: Consensus sequence strategy was used to design the novel and active antibacterial peptide named \'AGAAN\' from skin secretions of Agalychnis annae. The in-vitro activities of the novel peptide against some bacterial strains were investigated. Time kill studies, DNA retardation, cytotoxicity, betagalactosidase, and molecular computational studies were conducted.
RESULTS: AGAAN inhibited P. aeruginosa, E. faecalis, and S. typhimurium at 20 μM concentration. E. coli and S. aureus were inhibited at 25 μM, and lastly, B. subtilis at 50 μM. Kinetics of inactivation against exponential and stationary growing bacteria was found to be rapid within 1-5 hours of peptide exposure, depending on time and concentration. The peptide displayed weak hemolytic activity between 0.01%-7.31% at the antibacterial concentrations. AGAAN efficiently induced bacterial membrane damage with subsequent cell lysis. The peptide\'s DNA binding shows that it also targets intracellular DNA by retarding its movement. Our in-silico molecular docking analysis displayed a strong affinity to the bacterial cytoplasmic membrane.
CONCLUSIONS: AGAAN exhibits potential antibacterial properties that could be used to combat bacterial resistance.

Immune-mediated hemolytic anemia (IMHA) is an important cause of morbidity and mortality in dogs. IMHA also occurs in cats, although less commonly. IMHA is considered secondary when it can be attributed to an underlying disease, and as primary (idiopathic) if no cause is found. Eliminating diseases that cause IMHA may attenuate or stop immune-mediated erythrocyte destruction, and adverse consequences of long-term immunosuppressive treatment can be avoided. Infections, cancer, drugs, vaccines, and inflammatory processes may be underlying causes of IMHA. Evidence for these comorbidities has not been systematically evaluated, rendering evidence-based decisions difficult. We identified and extracted data from studies published in the veterinary literature and developed a novel tool for evaluation of evidence quality, using it to assess study design, diagnostic criteria for IMHA, comorbidities, and causality. Succinct evidence summary statements were written, along with screening recommendations. Statements were refined by conducting 3 iterations of Delphi review with panel and task force members. Commentary was solicited from several professional bodies to maximize clinical applicability before the recommendations were submitted. The resulting document is intended to provide clinical guidelines for diagnosis of, and underlying disease screening for, IMHA in dogs and cats. These should be implemented with consideration of animal, owner, and geographical factors.

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CONCLUSIONS: Daratumumab (DARA), a drug used to treat patients with multiple myeloma, causes interference in pre-transfusion testing. Samples from patients receiving DARA exhibit panreactivity in antibody detection and identification tests with red blood cells (RBCs). Many hospitals are sending these samples to reference laboratories. Dithiothreitol (DTT), a sulfhydryl chemical treatment of RBCs, negates this reactivity. This study investigated the stability of the antigens on DTT-treated RBCs to determine if large quantities of RBCs could be treated at one time, stored, and used for testing at a later time. Panel cells were treated with DTT and then stored as three sets. Set 1 DTT-treated RBCs were stored in Alsever\'s solution at 2°C to 8°C, washed daily, and suspended in pH 7.3 phosphate-buffered saline (PBS) prior to antigen typing. Set 2 DTT-treated RBCs were stored in pH 7.3 PBS. Set 3 DTT-treated RBCs were stored in Alsever\'s solution. Sets 2 and 3 were inspected daily for 14 days for observation of hemolysis. In Set 1, all antigen reactivity remained at ≥2+ with both single- and double-dose cells for 14 days. The Rh antigens gave stronger reactions longer, compared with those tested in the Duffy, Kidd, and MNS blood group systems. Sets 2 and 3 were monitored for hemolysis. On day 3, Set 2 began displaying hemolysis, with complete hemolysis by day 8. Set 3 did not display hemolysis in 14 days. In conclusion, a large volume of RBCs can be treated with DTT and stored in Alsever\'s solution for use without deterioration of the RBC antigens, saving institutions tech time, resources, and money.

Essentials An international collaboration provides a consensus for clinical definitions. This concerns thrombotic microangiopathies and thrombotic thrombocytopenic purpura (TTP). The consensus defines diagnosis, disease monitoring and response to treatment. Requirements for ADAMTS-13 are given.
Background Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic microangiopathy (TMA) is a broad pathophysiologic process that leads to microangiopathic hemolytic anemia and thrombocytopenia, and involves capillary and small-vessel platelet aggregates. The most common cause is disseminated intravascular coagulation, which may be differentiated by abnormal coagulation. Clinically, a number of conditions present with microangiopathic hemolytic anemia and thrombocytopenia, including cancer, infection, transplantation, drug use, autoimmune disease, and pre-eclampsia and hemolysis, elevated liver enzymes and low platelet count syndrome in pregnancy. Despite overlapping clinical presentations, TTP and HUS have distinct pathophysiologies and treatment pathways. Objectives To present a consensus document from an International Working Group on TTP and associated thrombotic microangiopathies (TMAs). Methods The International Working Group has proposed definitions and terminology based on published information and consensus-based recommendations. Conclusion The consensus aims to aid clinical decisions, but also future studies and trials, utilizing standardized definitions. It presents a classification of the causes of TMA, and criteria for clinical response, remission and relapse of congenital and immune-mediated TTP.

The American Society for Apheresis (ASFA) conducted a one-day consensus conference on red blood cell exchange (RBCx) in sickle cell disease (SCD) during its annual meeting in San Antonio, TX, on May 5, 2015. The authors of this article, a subcommittee of ASFA\'s Clinical Applications Committee, developed several questions with regard to pathophysiology of SCD and use of RBCx in the management of various complications. These questions were provided to the seven invited speakers who are the experts in the field of SCD. Two experts in the field moderated the proceedings of the conference, which was attended by more than 150 participants. After each presentation, there was a summary of the main points by the moderators and an open discussion with questions from the audience. A video recording of the proceedings, as well as each presentation, was made available to the authors. Each author\'s summary was reviewed and approved by the respective speaker before submission of this manuscript. The subcommittee also developed several key questions to generate a consensus amongst the speakers on key issues for using RBCx for patients with SCD.

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