Houston Methodist West Hospital, Houston, TX, USA.
Houston Methodist Hospital, Houston, TX, USA.
Am J Health Syst Pharm. 2022 Nov 7;79(22):1988-2000. doi: 10.1093/ajhp/zxac211.
This article aims to increase awareness of, outline pathophysiology for, and offer guidance on supportive care strategies for specific endocrine, neurological, and immunological syndromes associated with paraneoplastic syndromes (PNSs).
PNS refers to remote effects that cannot be attributed to the direct or invasive effects of a malignancy. These syndromes are considered clinically important because they may provide early recognition, diagnosis, and management of the malignancy in a timely manner. Many of their presenting symptoms such as ectopic Cushing's syndrome, hypercalcemia of malignancy (HCM), syndrome of inappropriate secretion of antidiuretic hormone (SIADH), neurological dysfunctions, and paraneoplastic autoimmune thrombocytopenia overlap with those of nonneoplastic disorders, yet their pathogenesis and responses to treatments differ. Management of ectopic Cushing's syndrome due to a PNS consists of treatment of the underlying malignancy and its comorbidities. Drug therapies may include ketoconazole, mitotane, metyrapone, somatostatin analogs, and dopamine agonists. Hypercalcemia may be classified into cases with parathyroid hormone (PTH)-dependent causes or PTH-independent causes such as HCM, in which osteoclast inhibitors may be deployed. Treatments of PNS-mediated SIADH include treatment of the underlying malignancy and strategies to increase serum sodium levels. Amifampridine is now considered the first-line agent for paraneoplastic Lambert-Eaton myasthenic syndrome, whereas steroids, intravenous immune globulin, thrombopoietin receptor agonists (eg, romiplostim, eltrombopag, and avatrombopag), fostamatinib, and rituximab may find their niche in treatment of PNS-mediated autoimmune thrombocytopenia.
Supportive care for PNSs lends opportunities to pharmacists to add quality, value, and safety.
本文旨在提高对副肿瘤综合征（PNS）相关特定内分泌、神经和免疫综合征的认识，概述其病理生理学，并提供支持性护理策略的指导。
PNS 是指无法归因于恶性肿瘤的直接或侵袭性影响的远处效应。这些综合征被认为具有临床重要性，因为它们可能及时提供恶性肿瘤的早期识别、诊断和管理。它们的许多表现症状，如异位库欣综合征、恶性肿瘤高钙血症（HCM）、抗利尿激素分泌不当综合征（SIADH）、神经功能障碍和副肿瘤性自身免疫性血小板减少症，与非肿瘤性疾病的表现症状重叠，但它们的发病机制和对治疗的反应不同。PNS 引起的异位库欣综合征的治疗包括治疗基础恶性肿瘤及其合并症。药物治疗可能包括酮康唑、米托坦、美替拉酮、生长抑素类似物和多巴胺激动剂。高钙血症可分为甲状旁腺激素（PTH）依赖性和 PTH 非依赖性，如 HCM，可使用破骨细胞抑制剂。PNS 引起的 SIADH 的治疗包括治疗基础恶性肿瘤和增加血清钠水平的策略。阿米福汀现在被认为是副肿瘤性 Lambert-Eaton 肌无力综合征的一线药物，而皮质类固醇、静脉注射免疫球蛋白、血小板生成素受体激动剂（如罗米司汀、芦可替尼和阿伐曲泊帕）、 fostamatinib 和利妥昔单抗可能在治疗 PNS 引起的自身免疫性血小板减少症中找到自己的位置。
PNS 的支持性护理为药剂师提供了增加质量、价值和安全性的机会。
