Institute for Neuro-Immune Medicine, Dr. Kiran C. Patel College of Osteopathic Medicine, Nova Southeastern University, Ft. Lauderdale, FL, USA.
Department of Immunology, Tufts University School of Medicine, Boston, MA, USA.
Expert Rev Clin Immunol. 2023 Jun;19(6):639-654. doi: 10.1080/1744666X.2023.2200936. Epub 2023 Apr 14.
Mast cells are found in all tissues and express numerous surface receptors allowing them to sense and respond to allergic, autoimmune, environmental, neurohormonal, pathogenic and stress triggers. Stimulated mast cells are typically called 'activated' but the mechanisms involved and the mediators released can vary considerably. Mast cell activation diseases (MCADs) include primary, secondary and idiopathic conditions, especially mast cell activation syndrome (MCAS), but mast cells are activated in many other disorders making the diagnosis and treatment challenging.
Mast cells can release numerous biologically active mediators, some of which are prestored in secretory granules while others are newly synthesized and released without degranulation. Most of the emphasis has so far been on secretion of histamine and tryptase, which do not explain all the multisystemic symptoms experienced by patients with MCADs. As a result, drug development has focused on antiproliferative therapy or blocking the action of individual mediators and not on inhibitors of mast cell activation.
Activated mast cells are involved in the pathogenesis of MCADs, but also in other disorders making appropriate diagnosis and treatment challenging. The definition of mast cell activation should be expanded beyond histamine and tryptase, with an emphasis on better detection and treatments.
肥大细胞存在于所有组织中，并表达许多表面受体，使它们能够感知和响应过敏、自身免疫、环境、神经激素、病原体和应激触发。受刺激的肥大细胞通常被称为“激活的”，但涉及的机制和释放的介质可能有很大差异。肥大细胞激活疾病（MCADs）包括原发性、继发性和特发性疾病，特别是肥大细胞激活综合征（MCAS），但肥大细胞在许多其他疾病中被激活，这使得诊断和治疗具有挑战性。
肥大细胞可以释放许多具有生物活性的介质，其中一些储存在分泌颗粒中，而另一些则是新合成并释放的，而无需脱颗粒。到目前为止，大多数重点都放在组胺和类胰蛋白酶的分泌上，而这些并不能解释 MCAD 患者经历的多系统症状。因此，药物开发的重点集中在抗增殖治疗或阻断单个介质的作用上，而不是肥大细胞激活的抑制剂上。
激活的肥大细胞参与 MCADs 的发病机制，但也参与其他疾病，使适当的诊断和治疗具有挑战性。肥大细胞激活的定义应该扩展到组胺和类胰蛋白酶之外，重点是更好地检测和治疗。
