PPT-Mast Cell Activation Disease

Current Concepts Lawrence B Afrin MD Division of Hematology Oncology amp Transplantation University of Minnesota Wellapalooza 2016 June 11 2016 Learning Objectives amp Disclaimers. Mast Cell and Basophil Activation and Mediator ReleaseMast cells and basophils release secretory products by either anaphylactic degranulation (“compound exocytosis”) or piecemeal degranulat non-AIDS Morbidity. Peter W. Hunt, MD. Associate Professor of Medicine. UCSF HIV/AIDS Division. A shift in focus…. T cell activation as a target for interventions in the pre-ART era. Monocyte activation and inflammation a target during treated HIV disease. Mast Cell and Basophil Activation and Mediator ReleaseMast cells and basophils release secretory products by either anaphylactic degranulation (“compound exocytosis”) or piecemeal degranulat disorders. Clonal hematopoiesis (stem cell disorder). Marrow hypercellularity. Overproduction of one or more cell lines (effective hematopoiesis). Exception: myelofibrosis. Differentiation nearly normal. Director. Division of Allergy and Clinical Immunology. St. Michael’s Hospital. University of Toronto. Treatment of Mast Cell Activation Syndrome in Patients with Postural Orthostatic Tachycardia Syndrome and Hereditary Connective Tissue Disorders. Ehlers-Danlos Syndrome, and . Mast Cell Activation Syndrome. Ingrid Cheung, Juan Guzman, Roberto Mendoza-. Londono. , Scott Walsh, Karan Gandhi, Peter Vadas. The ILC Ehlers Danlos & Chronic Pain Conference. J. Ochotná. The. . main. . functions. . of. . the. . immune. . system. Immune. . system. . belongs. to . the. basic . homeostatic. . mechanisms. . Defense. . Autotolerance. . Immune. . Cem Akin, MD, PhD Professor of Medicine University of Michigan Department of Internal Medicine Division of Allergy and Clinical Immunology Disclosures • Consultancies – Novartis ( Midostaurin tria Mast cells are the major effector cells of acuteypersensitivity reactions and allergic disease. They originate in bone marrow and migrate toipheral tissues,where they mature.They arenot normally found determination to help predict biologic behavior, as well as surgical AgNOR (previously referred to as Table 1. Overview of the most relevant prognostic information provided by routine diagnostic hi Dan Med J c h S TRAC T INTRODUCTION: i m pl icated in severa l phy sio l o g ica l reactions. Its a b i l it y react to di ff erent stimuli impacts a variety o f conditions s uc h as ast h ma, In 2008 Dr. Afrin started coming to understand that a newly recognized type of mast cell disease, now called mast cell activation syndrome (MCAS), was the underlying diagnosis in many patients he was seeing who were each suffering large assortments — quite different from one patient to the next — of chronic multisystem inflammatory illnesses of unclear cause. Dr. Afrin soon gained experience that MCAS is far more prevalent than the only mast cell disease previously known to medicine (the rare disease of mastocytosis) and that most MCAS patients, once accurately diagnosed, can eventually find significantly helpful medications targeted at the disease. The frequency and magnitude of the improvements Dr. Afrin has seen — even the relief that comes from finally having a unifying diagnosis other than psychosomatism — have spurred him to focus in this area, not only tending to the needs of his patients but also pursuing research to advance our understanding of the disease and helping to educate other professionals who in turn can help even more of the many people who have long been suffering not only the symptoms of the disease but also the natural concern of not understanding why one would be so unlucky to have acquired so many medical problems. As it turns out, such patients are not so unlucky and truly have just one root issue (and a very common one at that), which has the biological capability to develop, directly or indirectly, into most or all of their previously diagnosed problems. There is a great deal yet to learn about this, but even with just the present very limited understanding, the opportunity to diagnose and help patients with MCAS seems to be enormous and Dr. Afrin felt a description of the disease, written for the general public, might help lead some MCAS patients on a journey to diagnosis and improvement sooner rather than later. Dr. Afrin hopes this book will help people who might have, or do have, MCAS. A portion of the proceeds of purchases of this book will go to support research and education in this area. Diagnosis and Treatment. Leonard Weinstock, MD, FACG. Associate Professor of Clinical Medicine. Washington University School of Medicine in St. Louis. Disclosures. Off-label use of medications. No FDA-approved MCAS Rx. K. Hartmann. ,. 1,2. I. Alvarez-Twose. ,. 3. B. Myers. ,. 4. A. Hirdt. ,. 5,6. C. B. Livideanu. ,. 7. J. A. Bernstein. ,. 8. P. Lugar. ,. 9. . A. F. Whyte. ,. 10. J. Anderson. ,. 11. F. Ruëff.