Cytokine Storm Syndrome 2nd Edition

Cytokine Storm Syndromes, including HLH and MAS, are frequently fatal disorders, particularly if not recognized early and treated during presentation. The genetics of Cytokine Storm Syndromes are being defined with many of the risk alleles giving rise to mutations in the perforin-mediated cytolytic pathway used by CD8 cytotoxic T cells and natural killer cells. These are being studied using murine models. Up to 10% of the general population may carry risk alleles for developing Cytokine Storm Syndromes, and Cytokine Storm Syndromes are being increasingly recognized around the world in pediatric and adult hospitals. A variety of infectious, rheumatic, and oncologic triggers are commonly associated with Cytokine Storm Syndromes, but understanding this disorder is critical for all researchers and physicians to ensure timely and appropriate therapy. This second edition addresses all aspects of the disorder, from genetics, pathophysiology, and ongoing research, to clinical presentations, risk factors and treatment. New-to-this-edition features include dedicated chapters on severe COVID-19 CSS, and post-COVID CSS of multi-system inflammatory syndrome in children (MIS-C). In addition, novel topics including CSS associated with Castleman disease, pregnancy, therapeutics, transplantation, and cardiac bypass, as well as treatment with JAK inhibitors are addressed.
细胞因子风暴综合征（包括 HLH 和 MAS）通常是致命的疾病，尤其是在就诊期间未及早发现和治疗的情况下。细胞因子风暴综合征的遗传学正在被定义，许多风险等位基因会导致 CD8 细胞毒性 T 细胞和自然杀伤细胞使用的穿孔素介导的细胞溶解途径发生突变。这些正在使用小鼠模型进行研究。高达 10% 的普通人群可能携带患细胞因子风暴综合征的风险等位基因，并且细胞因子风暴综合征在全世界的儿科和成人医院中越来越得到认可。多种感染性、风湿性和肿瘤性触发因素通常与细胞因子风暴综合征有关，但了解这种疾病对于所有研究人员和医生确保及时和适当的治疗至关重要。第二版涉及该疾病的所有方面，从遗传学、病理生理学和正在进行的研究，到临床表现、危险因素和治疗。此版本的新功能包括有关严重 COVID-19 CSS 和儿童多系统炎症综合征 （MIS-C） 的 COVID 后 CSS 的专门章节。此外，还讨论了新的主题，包括与Castleman病、妊娠、治疗、移植和心脏搭桥相关的CSS，以及JAK抑制剂的治疗。