Department of General and Medical Biochemistry, University of Gdańsk, Gdańsk, Poland; Outdoor Clinic for Immunological Diseases of Children, Department of Pediatrics, Hematology and Oncology, Medical University of Gdańsk, Gdańsk, Poland.
Department of General and Medical Biochemistry, University of Gdańsk, Gdańsk, Poland.
Adv Med Sci. 2019 Mar;64(1):124-130. doi: 10.1016/j.advms.2018.08.013. Epub 2019 Jan 11.
The role of mast cell (MC) activity in pathophysiology is complex and challenging and its clinical effects are difficult to predict. Apart from the known role of MCs in basic immunological processes and allergy, underlined is their importance in bone mineralization and in regulation of autoimmune reactions. Mast cell mediators, especially those released from mast cells in degranulation, but also those released constitutively, are important both in metabolic and immunological processes. Mastocytosis is a heterogeneous group of disorders characterized by accumulation of MC in one or more organs. There are scientific data indicating that mastocytosis patients are at increased risk of osteoporosis in the systemic form of the disease and children with cutaneous mastocytosis have a higher rate of hypogammaglobulinemia. Moreover, the origin of osteoporosis in patients with allergy is no longer considered as linked to steroid therapy only, but to the mast cell mediators' activity as well. There are indications that osteoporosis symptoms in this group of patients may develop independently of the cumulative steroids' dose. Thus, the influence of mast cells on metabolic and immunologic processes in allergic patients should be investigated. The assessment of mast cell activity and burden in mastocytosis may be used to guide clinical management of patients with allergy.
肥大细胞(MC)活性在病理生理学中的作用复杂且具有挑战性,其临床效果难以预测。除了已知的 MC 在基础免疫过程和过敏中的作用外,MC 在骨矿化和自身免疫反应调节中的重要性也逐渐凸显。肥大细胞介质,特别是从脱颗粒肥大细胞释放的介质,以及组成性释放的介质,在代谢和免疫过程中都很重要。肥大细胞增多症是一组异质性疾病,其特征是一个或多个器官中 MC 的积累。有科学数据表明,肥大细胞增多症患者在全身性疾病中更容易发生骨质疏松症,患有皮肤肥大细胞增多症的儿童更易发生低丙种球蛋白血症。此外,过敏患者骨质疏松症的病因不再仅仅被认为与类固醇治疗有关,还与肥大细胞介质的活性有关。有迹象表明,这组患者的骨质疏松症症状可能与累积类固醇剂量无关。因此,应研究肥大细胞对过敏患者代谢和免疫过程的影响。肥大细胞增多症中肥大细胞活性和负荷的评估可用于指导过敏患者的临床管理。