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Указываемые данные об автономной и аллогенетической трансплантации гематопоэтики стем-целлов у взрослых: состояние искусства.

Пересадка стволовых клеток (ХХСТ) гематополиетическая трансплантация превратилась из процедуры спасания лейкемии в лечебную форму, охватывающую почти все виды гематологической злокачественности и несколько ненасыщенных расстройств. Современный ландшафт был видоизменен тремя совпадающими силами: усовершенствованием стратификации с учетом конкретных рисков заболеваний (European LeukemiaNet [ELN] 2022 для острой миелоидной лейкемии, Молекулярной международной системы прогнозирования (IPSS-M) для миэлодипластических синдромов, Международной системы мутационного скручивания [MIPSS70+ v.2.0] и Myelo

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… Paroxysmal Nocturnal Hemoglobinuria Complement inhibitors (the anti-C5 agents eculizumab and ravulizumab, the C3 inhibitor pegcetacoplan, and the oral factor B inhibitor iptacopan) have essentially eliminated allo-HSCT as front-line therapy in classical paroxysmal nocturnal hemoglobinuria cases [ 232 … Transplantation remains an option only for the aplasia/paroxysmal nocturnal hemoglobinuria syndrome with severe cytopenia (following SAA criteria), for evolution to a myeloid malignancy, or where complement inhibition is unavailable. 9.3. …

Biomarker Jeopardy.

In the popular Biomarker Jeopardy session, Sandra E. Kurtin, PhD, ANP-C, AOCN®, Alyssa Henglefelt, PharmD, BCOP, and Haleigh Mistry, MS, PA-C, paired biomarkers with tumor types for which their expression is most commonly used to determine targeted therapy, identified key assays used to measure common biomarkers, and discussed guidelines for biomarker testing.

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… Paroxysmal nocturnal hemoglobinuria (PNH) is a rare complement driven non-immune hemolytic anemia that is driven by complement inhibitors on CD55 and CD59 that converge at C3 convertase. Pegcetacoplan (Empaveli) is a C3-based therapeutic combining APL-2 and pegcetacoplan. …

Continuing cancer care delivery during the peak of COVID-19 in the Bronx, New York: experience from a public teaching hospital.

IntroductionWe report our experience with cancer care delivery during the peak of COVID-19 pandemic in New York City.MethodsRetrospective analysis of the patients treated from the 1st of March, 2020 to the 8th of May, 2020.ResultsTeam huddles, infection screening and patient selection strategies were implemented. One hundred and seventy patients were treated in 576 visits. Six developed severe COVID-19 requiring hospitalization, two died. Their median Charlson Comorbidity Index was 9, higher than the rest of the cohort.ConclusionsCancer care delivery is safe and feasible using an approach focu

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… Five patients with non-malignant hematological conditions were also treated, including 1 patient with hemophagocytic lymphohistiocytosis, 1 with paroxysmal nocturnal hemoglobinuria, 2 with myelodysplastic syndrome and 1 with severe vitamin B 12 deficiency. …

Eosinophilic fasciitis (Shulman's disease): review and comparative evaluation of seven patients.

ObjectivesEosinophilic fasciitis (EF) was described in 1974 by Shulman as a rare fibrosing connective tissue disease of unknown etiology. An undetermined trigger is thought to lead to the degranulation of eosinophils that interact with fibroblasts and express fibrogenic cytokines including the transforming factor of tumor growth a and b and interleukins 1 and 6. The purpose of this study was to summarize seven cases of EF in a central hospital.Material and methodsThis was a retrospective and descriptive study of a population with EF of a central hospital. All patients diagnosed with EF in a ho

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… Hematological abnormalities associated with EF include the following: multiple myeloma, lymphoma, myelodysplastic syndromes, myeloproliferative disorders, acquired amegakaryocytic thrombocytopenia, aplastic anemia, and paroxysmal nocturnal hemoglobinuria. …

Precision medicine and monoclonal antibodies: breach of promise?

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… disease, psoriatic arthritis, psoriasis, ankylosing spondylitis Adalimumab | 2003 | idiopathic arthritis, plaque psoriasis, Crohn's disease, uveitis Omalizumab | 2005 | asthma Natalizumab | 2006 | multiple sclerosis Ranibizumab | 2007 | macular degeneration Eculizumab | 2007 | paroxysmal nocturnal hemoglobinuria …

New and Emerging Biological Therapies for Myasthenia Gravis: A Focussed Review for Clinical Decision-Making.

Myasthenia gravis (MG) is a rare autoimmune disease characterised by exertion-induced muscle weakness that can lead to potentially life-threatening myasthenic crises. Detectable antibodies are directed against specific postsynaptic structures of the neuromuscular junction. MG is a chronic condition that can be improved through therapies, but to date, not cured. Standard treatment has been unchanged for decades and includes symptomatic treatment with acetylcholine-esterase inhibitors and disease-modifying treatment with steroids, steroid-sparing immunosuppressants and thymectomy. Overall, a rel

The risk of cytomegalovirus infection in non-myeloablative peripheral stem cell transplantation compared with conventional bone marrow transplantation.

Non-myeloablative allogeneic peripheral stem cell transplantation (NST) is a novel therapeutic strategy for patients with hematologic malignancies. Whether non-myeloablative transplants are associated with increased risk of cytomegalovirus (CMV) infections is unknown. To clarify this issue, we compared the outcome of CMV infection following 24 allogeneic non-myeloablative peripheral blood stem cell transplants and 40 conventional bone marrow transplants (CBT). The NST regimen consisted (mg/kg). Twelve patients (50%) in the NST group and 17 (43%) in the CBT group developed positive antigenemia

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… . * Others included Non-Hodgkin's lymphoma (n=6), Hodgkin's disease (n=3), multiple myeloma (n=3), paroxysmal nocturnal hemoglobinuria (n=2) in NST group, and one case of non-Hodgkin's lymphoma in CBT group. …

16th International Congress on Antiphospholipid Antibodies Task Force Report on Antiphospholipid Syndrome Treatment Trends.

Antiphospholipid syndrome (APS), an acquired autoimmune thrombophilia, is characterised by thrombosis and/or pregnancy morbidity in association with persistent antiphospholipid antibodies. The 16th International Congress on Antiphospholipid Antibodies Task Force on APS Treatment Trends reviewed the current status with regard to existing and novel treatment trends for APS, which is the focus of this Task Force report. The report addresses current treatments and developments since the last report, on the use of direct oral anticoagulants in patients with APS, antiplatelet agents, adjunctive ther

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… Outcomes in complement-mediated thrombotic microangiopathies (TMAs), such as paroxsysmal nocturnal hemoglobinuria (PNH) and atypical haemolytic uremic syndrome (aHUS), were improved with anti-complement therapy (anti-C5). …

Influenza A H1N1 Triggering Complement-Mediated Hemolytic Uremic Syndrome.

Complement-mediated hemolytic uremic syndrome (CM-HUS) is a condition characterized by hemolytic anemia, thrombocytopenia, and renal impairment, typically associated with a chronic dysregulation of the complement system. Few adult cases of CM-HUS triggered by influenza infection have been described. Here we present a case of a 56-year-old female with multiple myeloma receiving cancer treatment who presented with Influenza A H1N1-associated CM-HUS. She was treated with anti-complement therapy and at her 1-month follow-up had complete resolution of renal dysfunction and hemolytic anemia. This ca

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… Real-world safety profile of eculizumab in patients with paroxysmal nocturnal hemoglobinuria, atypical hemolytic uremic syndrome, or generalized myasthenia gravis: an integrated analysis of post-marketing surveillance in Japan. …

Immunotherapeutic approaches for systemic lupus erythematosus: early overview and future potential.

Systemic lupus erythematosus (SLE) is a complex autoimmune disease. Current SLE therapies include immunosuppressants, antimalarial drugs, non-steroidal anti-inflammatory drugs (NSAIDs), and corticosteroids, but these treatments can cause substantial toxicities to organs and may not be effective for all patients. In recent years, significant progress has been made in the treatment of SLE using immunotherapy, including Benlysta and Saphnelo. These advances in immunotherapy hold promise for SLE patients, providing new therapeutic options that may offer better clinical benefit and effectiveness. S

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… According to news provided by Kira Pharmaceuticals, KP-104 therapy is recently granted orphan drug designation by FDA for treatment of paroxysmal nocturnal hemoglobinuria (PNH). A completed phase I first-in-human (FIH) study had shown the prominent clinical proof-of-mechanism (POM). … BCDA, blood dendritic cell antigen; CD, cluster of differentiation; DLE, discoid lupus erythematosus; ILT, immunoglobulin-like transcript; LN, lupus nephritis; PNH, paroxysmal nocturnal hemoglobinuria; SLE-TM, SLE associated with thrombotic microangiopathy. …

IgA Nephropathy: Beyond the Half-Century.

In 1968, Jean Berger first introduced the medical world to IgA nephropathy (IgAN). Fifty-five years later, its pathogenesis is still unclear, but treatments such as renin-angiotensin-aldosterone system inhibitors (RAAS-Is), tonsillectomies, and glucocorticoids are currently used worldwide. There have been great strides in the past 20 years since the discoveries of the specific dysregulation of mucosal immunity, galactose-deficient IgA1 (Gd-IgA1), and Gd-IgA1 immune complexes in patients with IgAN. According to these findings, a multi-hit hypothesis was developed, and this multi-hit hypothesis

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… It was initially developed for the treatment of complement-mediated paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) [ 47 ]. Eculizumab, which selectively binds to C5, prevents the formation of C5a and MAC by inhibiting C5 cleavage. … Severe Infection of Pseudomonas aeruginosa during Eculizumab Therapy for Paroxysmal Nocturnal Hemoglobinuria. Int. Med. 2018;57:127–130. doi: 10.2169/internalmedicine.9151-17. 49. Hawkins K.L., Hoffman M., Okuyama S., Rowan S.E. A Case of Fulminant Meningococcemia: It Is All in the Complement. …

Skin manifestations associated with systemic diseases - Part I.

The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes

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… Gastrointestinal tract Ulcerative colitis Crohn’s disease Chronic active hepatitis Regional enteritis Primary biliary cirrhosis Endocrinological Diabetes mellitus Thyroid diseases Others Auto-inflammatory syndromes with: Suppurative hidradenitis; Neutrophilic dermatoses; Acne conglobate Paroxysmal nocturnal hemoglobinuria … Gastrointestinal tract | Ulcerative colitis Crohn’s disease Chronic active hepatitis Regional enteritis Primary biliary cirrhosis Endocrinological | Diabetes mellitus Thyroid diseases Others | Auto-inflammatory syndromes with: Suppurative hidradenitis; Neutrophilic dermatoses; Acne conglobate Paroxysmal nocturnal hemoglobinuria …

Immunosuppression in Glomerular Diseases: Implications for SARS-CoV-2 Vaccines and COVID-19.

BackgroundGlomerular diseases (GD) are chronic conditions that often involve immune dysfunction and require immunosuppressive therapy (IST) to control underlying pathogenesis. Unfortunately, such diseases appear to heighten risks of severe outcomes in COVID-19 and predispose to other infections that may be life-threatening. Thus, averting preventable infections is imperative in GD patients.SummaryThe advent of vaccines demonstrated to be safe and efficacious against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has favorably impacted the COVID-19 pandemic epidemiology. However,

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… This concept is supported by the observation that response to MenACWY vaccine in paroxysmal nocturnal hemoglobinuria was significantly better than that observed in aHUS patients [ 85 ]. … patients with atypical haemolytic uraemic syndrome treated with eculizumab Nephrol Dial Transpl 2020 35 (2) 298 303 10.1093/ndt/gfy225 29992261 85 Alashkar F Vance C Herich-Terhürne D Preising N Dührsen U Röth A Serologic response to meningococcal vaccination in patients with paroxysmal nocturnal hemoglobinuria …

Novel Treatments in Myasthenia Gravis.

Myasthenia gravis (MG) is the prototypical autoimmune disorder caused by specific autoantibodies at the neuromuscular junction. Broad-based immunotherapies, such as corticosteroids, azathioprine, mycophenolate, tacrolimus, and cyclosporine, have been effective in controlling symptoms of myasthenia. While being effective in a majority of MG patients many of these immunosuppressive agents are associated with long-term side effects, often intolerable for patients, and take several months to be effective. With advances in translational research and drug development capabilities, more directed ther

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… It was initially approved by the Food and Drug Administration (FDA) for the treatment of paroxysmal nocturnal hemoglobinuria in 2007 and has since then been approved for use in atypical haemolytic uraemia, generalised MG and neuromyelitis optica ( Table 1 ) ( 40 , 41 ). … Ravulizumab: a novel c5 inhibitor for the treatment of paroxysmal nocturnal hemoglobinuria . Ther Adv Hematol. ( 2019 ) 10 : 2040620719874728 . 10.1177/2040620719874728 31534662 PMC6737867 49. Kulasekararaj AG Hill A Rottinghaus ST Langemeijer S Wells R Gonzalez-Fernandez FA . …

Use of complementary and alternative medicine by patients with hematological diseases experience at a university hospital in northeast Mexico.

BackgroundComplementary and alternative medicine includes a diverse group of medical and healthcare systems, practices and products not considered part of conventional medicine. Although there is information on unconventional practices in oncological diseases, specific data regarding the use of complementary and alternative medicine by hematology patients is scarce.ObjectiveThe aim of this study is to document the prevalence of this modality of unconventional therapy in patients with malignant and benign hematological diseases, particularly children with acute lymphoblastic leukemia.MethodsAn

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… Gender M/F | Age-median years | | Adults n (%) | Children n (%) Autoimmune hemolytic anemia | 1/3 | 1/3 | | 3 21.4 | 1 50.0 Aplastic anemia; | 0/2 | 0/2 | | 1 7.1 | 1 50.0 Idiopathic thrombocytopenic purpura | 3/5 | 3/5 | | 8 57.1 | - - Hemophilia | 0/1 | 0/1 | | 1 7.1 | - - Paroxysmal nocturnal hemoglobinuria …

Advances in drug therapy for systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disorder that afflicts 500,000 people in the United States. There has not been a new SLE drug approved in the United States since 1958. However, a guidance document issued by the Food and Drug Administration in 2005 provided a roadmap for investigators which spawned numerous ongoing clinical trials. Among these, Belimumab, a monoclonal antibody to soluble B lymphocyte stimulator, met its primary endpoints in two large trials and will probably obtain FDA approval soon. Other promising agents targeting a variety of mechanisms of action are curr

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… Innate immunity, including complement Monoclonal antibodies to C5a were studied and shown to be safe in a phase I trial a decade ago with eculizumab (Solaris/Alexion, Cheshire, CT, USA), an agent now available for paroxysmal nocturnal hemoglobinuria [ 28 ]. …

Treatment of oral mucositis in hematologic patients undergoing autologous or allogeneic transplantation of peripheral blood stem cells: a prospective, randomized study with a mouthwash containing camelia sinensis leaf extract.

Oral mucositis is an important side effect of hematopoietic stem cell transplantation (HCST), mainly due to toxicity of conditioning regimens. It produces significant pain and morbidity. The present study reports a prospective, randomized, non-blinded study testing the efficacy of a new mouthwash, called Baxidil Onco(®) (Sanitas Farmaceutici Srl, Tortona, Italy) in 60 hematologic patients undergoing HCST (28 autologous, 32 allogeneic). Baxidil Onco(®), used three times a day from Day -1 to Day +30, in addition to standard prophylactic schedules, was administered to 14 patients undergoing autol

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… suffering from: multiple myeloma (MM, n=23), B-cell non-Hodgkin’s lymphomas (NHL, n=14), acute myeloblastic leukemia (AML, n=10), acute lymphoblastic leukemia (ALL, n=7), chronic lymphocytic leukemia (CLL, n=2), primary myelofibrosis (PMF, n=1), myelodysplastic syndrome (MDS, n=1), paroxysmal nocturnal hemoglobinuria …

Клиническая реакция и переливание овечек, подвергшихся однополому переливанию крови.

Исследования, касающиеся сохранения крови и реагирования на переливание крови, недостаточны для румын. Мы провели оценку клинических проявлений овец, которые получили одно гомологическое переливание целой крови, сосредоточившись на реакциях на переливание. Eighteen Взрослые овцы подвергались одной флеботомии, с тем чтобы избавиться от нее. 40% общего объема крови, который был помещен в CPDA-1 мешки, а затем разделённые на G0, животные, которые получили свежую кровь, и G15 и G35, животные, которые получали кровь, хранимую для 15 или 35 Дни, соответственно. В ходе переливания были зарегистрирова

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… Hunt and Moore [ 7 ] considered that the commonest signs of transfusion reactions in ruminants are tachycardia, tachypnea, sudoresis, tremors, fever, pruritus, dyspnea, hematuria, and hemoglobinuria, among others. …

Effects of Exercise Programs on Psychoemotional and Quality-of-Life Factors in Adult Patients with Cancer and Hematopoietic Stem Cell Transplantation or Bone Marrow Transplantation: A Systematic Review.

This review analyzed the effects of an exercise program on psychoemotional and quality-of-life (QoL) factors in adult patients with cancer and hematopoietic stem cell transplantation (HSCT) or bone marrow transplantation (BMT). Studies were identified from the PubMed and Web of Science databases (from inception to 24 August 2022), according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) checklist. The methodological quality of the included studies was assessed with the Physiotherapy Evidence Database (PEDro) scale, based in turn on the Delphi list. A total o

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… = Hematopoietic stem cell transplantation; HRmax = Heart rate maximal; MDS = Myelodysplastic síndrome; MF = Myelofibrosis; MFI = multidimensional fatigue inventory; MM = Múltiple mieloma; MPS = Myeloproliferative síndrome; PF = Physical functioning; Pf = Physical fatigue; PNH = paroxysmal nocturnal hemoglobinuria … Montgomery–Âsberg Depression Rating Scale; MDS = myelodysplastic syndrome; MFI = Multidimensional Fatigue Inventory; MIP = maximal inspiratory pressure; MM = multiple myeloma; MMRC = Modified Medical Research Council; MSEC = maximal short exercise capacity; PF = physical functioning; PNH = paroxysmal nocturnal hemoglobinuria …

Histiocytoid giant cellulitis-like Sweet syndrome at the site of sternal aspiration: A case report and review of literature.

BackgroundGiant cellulitis-like Sweet syndrome (SS) is a rare subtype of SS, and reports of the combined histiocytoid type of pathology are scarce. Here, we report a case of SS with distinctive clinical presentations and which was difficult to distinguish from cellulitis. By sharing this case and a discussion of the related literature in detail, we aim to provide clinicians with new insights into the characteristics of histiocytoid giant cellulitis-like (HGC)-SS and the pathogenesis of SS.Case summaryA 52-year-old male was admitted after experiencing progressive fatigue for 1 mo and tongue swe

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… Direct antiglobulin (Coombs) testing and paroxysmal nocturnal hemoglobinuria clone testing yielded negative results. There was high suspicion of hematologic malignancy because of pancytopenia, splenomegaly, and increased blast cell count in PB. …