Autoimmune Diseases Unit, Research and Innovation Group in Autoimmune Diseases, Sanitas Digital Hospital, Hospital-CIMA-Centre Mèdic Milenium Balmes Sanitas, Barcelona, Spain.
SarcoGEAS Study Group ("Grupo de Estudio de Enfermedades Autoinmunes -GEAS-, Sociedad Española de Medicina Interna -SEMI-), Spain.
Expert Rev Clin Immunol. 2024 Jan-Jun;20(1):59-70. doi: 10.1080/1744666X.2023.2274363. Epub 2024 Jan 8.
We present an updated overview of the hematological involvementassociated with sarcoidosis, including a management approach forcytopenias and revisiting the association with hematologicalmalignancies.
Theetiology of cytopenias in sarcoidosis can be attributed to two majoretiopathogenic mechanisms: infiltration of hematopoietic organs suchas the spleen and bone marrow, and autoimmune-mediated cytopenias.With respect to the association with hematological malignancies, itrequires careful evaluation of patients from a chronologicalperspective. Patients must be classified into one of three pathogenicscenarios, including preexisting hematological malignancies,synchronous development of malignancy and sarcoidosis due to commonpredisposing factors, or sarcoidosis as a predisposing factor formalignancies.
The association between sarcoidosis and hematologic involvement isbest understood as a pathogenic continuum, with cytopenias andhematologic neoplasms intertwined due to various etiopathogenicmechanisms. These mechanisms include sarcoid infiltration ofhematopoietic organs, common predisposing immunogenetics for thedevelopment of autoimmune cytopenias and malignancies, and anincreased risk of neoplasm development in patients with autoimmunecytopenias. Collaboration among the main specialties involved in theclinical management of these patients is crucial for an earlymonitoring and management.
我们对与结节病相关的血液学受累进行了更新概述，包括血细胞减少症的治疗方法，并重新探讨了与血液系统恶性肿瘤的关联。
结节病引起血细胞减少的病因可归因于两个主要的发病机制：造血器官（如脾脏和骨髓）的浸润，以及自身免疫性血细胞减少症。关于与血液系统恶性肿瘤的关联，需要从时间顺序上仔细评估患者。患者必须分为三种发病机制之一，包括先前存在的血液系统恶性肿瘤、恶性肿瘤与结节病同时发生，这是由于共同的易感因素所致，或结节病是恶性肿瘤的易感因素。
结节病和血液学受累之间的关联最好被理解为一个发病连续体，血细胞减少症和血液系统肿瘤由于各种发病机制而交织在一起。这些机制包括结节病对造血器官的浸润、自身免疫性血细胞减少症和恶性肿瘤发展的共同易感免疫遗传学，以及自身免疫性血细胞减少症患者发生肿瘤的风险增加。参与这些患者临床管理的主要专业之间的合作对于早期监测和管理至关重要。
