Transfusion Medicine Reviews

Labeling of platelets (PLTs) is essential for research purposes, in order to measure the recovery and survival of transfused PLTs in vivo. Biotinylation is a promising new alternative to the gold standard of radioactive labeling. This review highlights 4 key publications that provide significant insights into biotin-labeled PLTs (bioPLTs). Stohlawetz et al. established that transfusion of bioPLTs in human recipients is possible. De Bruin et al. developed a standardized, reproducible protocol for biotinylation of PLTs as a promising method to trace and isolate transfused PLTs in vivo, with reduced levels of PLT activation markers...

RBC alloimmunization remains a significant barrier to ongoing transfusion therapy leading to morbidity, and in extreme cases mortality, due to delayed or insufficient units of compatible RBCs. In addition, the monitoring and characterization of alloantibodies, often with multiple specificities in a single patient, consumes substantial health care resources. Extended phenotypic matching has mitigated, but not eliminated, RBC alloimmunization and is only logistically available for specialized populations. Thus, RBC alloimmunization remains a substantial problem...

Therapeutic apheresis refers to a diversity of procedures in which specific hematologic components (e.g., plasma, erythrocytes, leukocytes, etc.) with pathological associations are removed from circulation (with possible replacement) in order to treat a variety of disease processes. As pharmacologic agents also circulate with these components, their removal is sometimes incidental, or in the scenario of drug toxicity, a therapeutic goal. The corpus of published manuscripts on this subject has grown immensely over the past few decades; however, the breadth of diseases, methods, and drugs that co-exist in this space make it challenging to generate generalizable evidence regarding drug removal via apheresis...

No abstract text is available yet for this article.

Blood collection from minority populations improves the transfusion support of patients with sickle cell disease and thalassemia, but efforts are challenged by high deferral rates for hemoglobin (Hb) eligibility thresholds. This study sought to evaluate hemoglobin and iron status of a representative US female population to assess the suitability of 12.0 g/dL as minimum hemoglobin. Data were extracted from the National Health and Nutrition Examination Surveys (NHANES), 1999-2010. A national sample designed to reflect potential female blood donors (weight ≥110 lbs, not pregnant, no infectious marker reactivity, and no blood donation in past year) aged 16 to 49 years was analyzed for Hb and serum ferritin (SF) measures by race/ethnicity (N = 6937)...

Blood Banking/Transfusion Medicine (BB/TM) specialists oversee all aspects of blood component transfusions and are often involved with apheresis, coagulation, and cellular therapy services as well. This study characterizes the BB/TM workforce to determine the scholarly productivity in the first 3 to 5 years after obtaining board certification and the impact of job type, job location, and academic rank on scholarly productivity. Academic productivity was assessed among individuals passing the American Board of Pathology BB/TM board exam between 2016 and 2018 using the National Institutes of Health (NIH) Office of Portfolio Analysis tool, iCite...

Therapeutic plasma exchange (TPE) has traditionally been used to selectively remove pathologic contents including autoantibodies, abnormal proteins, immune complexes, or toxins from a patient's plasma. In addition to the removal of molecular contributors to disease, fluid replacement and infusion of beneficial plasma constituents including albumin can be tapered based on the pathophysiologic mechanisms of the offending disease. This treatment modality has shown efficacy in symptomatic relief and slowing of disease progression for various neurologic, immunologic, and hematologic diseases...

Immune thrombotic thrombocytopenic purpura (iTTP) is a microangiopathic hemolytic anemia (MAHA) underpinned by autoreactivity against the von Willebrand factor (vWF) cleaving protease, ADAMTS13 (adisintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13). Autoantibody mediated ADAMTS13 inhibition leads to the accumulation of ultra-large vWF multimers which activate platelets and endothelium to initiate microvascular thrombosis. In the absence of urgent therapeutic intervention, iTTP is rapidly fatal due to cumulative organ dysfunction including catastrophic neurological and cardiac sequalae...

Autoreactive B-cells are crucial in the pathogenesis of both haematologic and non-haematologic autoimmune disorders. Therapies targeting B cells and autoantibodies are widely used in clinical practice, however, many patients fail to respond to conventional treatments. An evolving understanding of molecular mechanisms underlying autoimmune haematologic disorders has facilitated the development of novel therapies, including Bruton's Tyrosine Kinase (BTK) inhibitors. BTK is fundamental in B-cell survival, and its inhibition has been used in a wide range of autoimmune and inflammatory conditions, as well as mature B cell malignancies...

Acquired Hemophilia A (AHA) is a rare, life-threatening bleeding disorder from autoantibodies against clotting factor VIII. These autoantibodies occur with increasing incidence with advanced age and are often associated with other medical conditions such as autoimmune diseases and malignancy. Not uncommonly, AHA presents as a new bleeding disorder in a person with prior thrombosis or thrombotic risk. Treatment of AHA focuses on managing and preventing bleeding, as well as immunosuppression with the goal to eradicate the autoantibody...

The antiphospholipid syndrome (APS) is the most common cause of acquired immune-mediated thrombophilia. This syndrome is broadly defined by the presence of arterial or venous thrombosis, or pregnancy morbidity, in the presence of high levels of antiphospholipid antibodies. Despite recognition of this disorder more than 50 years ago, a fundamental unifying pathogenesis has not been determined. Due to this, mechanism-based therapies for APS are not available, and current management following thrombotic events suggests anticoagulation of indeterminate duration, or for obstetric complications, heparin/low molecular weight heparin and aspirin...

Autoimmune neuropathies are often treatable. First-line immunotherapies include intravenous immunoglobulin (IVIG), plasma exchange and corticosteroids. However, nearly 15-30% of patients are either refractory, partially responsive or chronically dependent on these first-line agents. Lack of full response leads to increased disability in addition to adverse financial implications. Consequently, there is an unmet need for more effective treatments to manage this subset of patients. There has been a remarkable increase in the knowledge about immunopathogenesis, antigenic targets, clinical phenotype correlation, and novel therapeutic agents in the last two decades...

No abstract text is available yet for this article.

Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis comprises a rare entity of disorders that affects primarily small and medium-sized blood vessels. Since first documented in 1897, we have come a long way trying to understand the pathogenesis and finding an optimal treatment regimen. The pathogenesis of ANCA vasculitis is not well understood and despite many advances in treatment, the morbidity and mortality remains high. Over the last decade, there have been many advancements toward elucidating the pathogenesis, optimizing current therapies, and discovering new medicines...

In this review article we provide a critical insight into recent reports evaluating innovative therapies for warm type autoimmune hemolytic anemia (wAIHA). Among published articles, we selected two reports on the use of the proteasome inhibitor bortezomib in association with dexamethasone or rituximab, one study on the spleen tyrosine kinase inhibitor fostamatinib, and a retrospective study on recombinant erythropoietin (rEPO). Among recent scientific communications, we discussed a report on the phosphoinositide 3-kinase delta inhibitor (PI3Kδi) parsaclisib...

Neuromyelitis optica spectrum disorder (NMOSD) is a chronic autoimmune disease of the central nervous system, characterized by recurrent attacks of optic neuritis, transverse myelitis, brainstem, and/ or cerebral symptoms. Despite the current standard of care consisting of high-dose corticosteroids and therapeutic plasma exchange, many patients are left with a permanent neurological disability after each attack. With the recent advancements in understanding the pathogenic mechanisms involved in NMOSD relapses, possibilities to develop new targeted therapies are anticipated...

Two major steps are identified in the pathogenesis of cold agglutinin disease; clonal B-cell lymphoproliferation and complement-mediated hemolysis. Each of these steps constitutes a target for treatment. In this focused review, we address 2 successful therapeutic approaches; the bendamustine plus rituximab combination as a highly efficacious B-cell directed therapy and the anti-C1s monoclonal antibody sutimlimab as the most extensively studied complement-targeting therapy. We describe and discuss the prospective study of bendamustine plus rituximab and 2 recent, prospective studies of sutimlimab...

No abstract text is available yet for this article.

Immune thrombocytopenia (ITP) is an autoimmune disease characterized by isolated thrombocytopenia that may be accompanied clinically by bleeding and reduced health-related quality of life (HRQoL). While corticosteroids, splenectomy, and various immunosuppressants (used off-label) have served as historical mainstays of ITP treatment, their use is associated with adverse effects and morbidity. Over the last 15 years, the advent of the thrombopoietin receptor agonists has revolutionized the management of chronic ITP with high response rates, durable responses, and minimal adverse effects in most patients...

The discovery of bacterial enzymes with specificity for IgG antibodies has led to breakthroughs in several autoantibody-mediated diseases. Two such enzymes, IdeS and EndoS, degrade IgG by different mechanisms, and have separately shown promise in numerous animal models of autoimmune diseases. Recently, imlifidase (the international nonproprietary name for IdeS) has advanced to clinical trials, where it has performed remarkably well in desensitizing patients to enable kidney transplantation, and in anti-glomerular basement membrane disease...