PDF(Pubmed)
Abstract：
UNASSIGNED: Castleman disease (CD) is a rare lymphoproliferative disorder with various subtypes, including the HHV-8-negative/idiopathic multicentric CD (iMCD). The diagnosis of iMCD remains challenging due to its non-specific presentation, in the form of generalised lymphadenopathies and inflammation. Two clinical presentations have been recently defined: a severe form iMCD-TAFRO and a milder form of iMCD not otherwise specified (iMCD-NOS). identification of interleukin-6 (IL-6) as a major culprit of inflammatory symptoms led to the development of anti-IL-6 therapies, with siltuximab being the approved first-line treatment.
UNASSIGNED: A 16-year-old male presented with recurrent fever, night sweats and several other non-specific symptoms. After extensive evaluations, an excisional lymph node biopsy confirmed the iMCD-NOS diagnosis. The patient received high-dose steroid therapy followed by siltuximab for four years. This treatment was well tolerated with only mild neutropenia not leading to dose adjustment. On siltuximab, the patient developed two mild COVID-19 episodes. His response to siltuximab remained effective throughout four years.
UNASSIGNED: The absence of biomarker or causal agent identification poses a diagnostic challenge requiring lymph node histopathology for a definitive diagnosis of iMCD. Anti-IL 6 (siltuximab) is the recommended frontline therapy, suppressing inflammation and halting disease progression. Intravenous administration every 3 to 6 weeks can impact patient quality of life, prompting further research for alternative treatments. High-dose steroids, rituximab, cyclosporine, tacrolimus, lenalidomide or combined chemotherapy such as rituximab-bortezomib-dexamethasone are among the considered options according to disease severity.
UNASSIGNED: Overall, long-term siltuximab effectively controlled iMCD symptoms and was well tolerated by this young adult, who endured two mild COVID-19 episodes.
CONCLUSIONS: Lymph node biopsy rather than bone marrow biopsy is needed for the diagnosis of iMCD.We were able to control the patient\'s condition in the absence of cumulative toxicity during four years of siltuximab anti-IL6 therapy.Immunosuppressive anti-IL6 therapy did not worsen two episodes of COVID-19.
UNASSIGNED: A 16-year-old male presented with recurrent fever, night sweats and several other non-specific symptoms. After extensive evaluations, an excisional lymph node biopsy confirmed the iMCD-NOS diagnosis. The patient received high-dose steroid therapy followed by siltuximab for four years. This treatment was well tolerated with only mild neutropenia not leading to dose adjustment. On siltuximab, the patient developed two mild COVID-19 episodes. His response to siltuximab remained effective throughout four years.
UNASSIGNED: The absence of biomarker or causal agent identification poses a diagnostic challenge requiring lymph node histopathology for a definitive diagnosis of iMCD. Anti-IL 6 (siltuximab) is the recommended frontline therapy, suppressing inflammation and halting disease progression. Intravenous administration every 3 to 6 weeks can impact patient quality of life, prompting further research for alternative treatments. High-dose steroids, rituximab, cyclosporine, tacrolimus, lenalidomide or combined chemotherapy such as rituximab-bortezomib-dexamethasone are among the considered options according to disease severity.
UNASSIGNED: Overall, long-term siltuximab effectively controlled iMCD symptoms and was well tolerated by this young adult, who endured two mild COVID-19 episodes.
CONCLUSIONS: Lymph node biopsy rather than bone marrow biopsy is needed for the diagnosis of iMCD.We were able to control the patient\'s condition in the absence of cumulative toxicity during four years of siltuximab anti-IL6 therapy.Immunosuppressive anti-IL6 therapy did not worsen two episodes of COVID-19.
摘要：
■Castleman病（CD）是一种罕见的淋巴增生性疾病，具有多种亚型，包括HHV-8阴性/特发性多中心CD（iMCD）。由于其非特异性表现，iMCD的诊断仍然具有挑战性，以广泛性淋巴结病和炎症的形式。最近已经定义了两种临床表现:严重形式的iMCD-TAFRO和未另外指定的较温和形式的iMCD(iMCD-NOS)。白细胞介素-6（IL-6）作为炎症症状的主要罪魁祸首的鉴定导致了抗IL-6治疗的发展，siltuximab是批准的一线治疗。
■一名16岁男性出现反复发烧，盗汗和其他一些非特异性症状。经过广泛的评估，切除淋巴结活检证实了iMCD-NOS的诊断。患者接受高剂量类固醇治疗，随后接受siltuximab治疗四年。该治疗具有良好的耐受性,仅轻度中性粒细胞减少症不导致剂量调整。在siltuximab上，患者出现2次轻度COVID-19发作.他对siltuximab的反应在四年中仍然有效。
■缺乏生物标志物或病原体鉴定提出了诊断挑战，需要淋巴结组织病理学来明确诊断iMCD。抗IL6（siltuximab）是推荐的一线治疗，抑制炎症和停止疾病进展。每3至6周静脉给药可影响患者的生活质量，促使对替代疗法的进一步研究。高剂量类固醇,利妥昔单抗,环孢菌素,他克莫司,来那度胺或联合化疗如利妥昔单抗-硼替佐米-地塞米松是根据疾病严重程度考虑的选择之一.
■总的来说，长期的siltuximab有效地控制了iMCD症状，并且该年轻人耐受性良好，他经历了两次轻微的COVID-19发作。
结论：诊断iMCD需要淋巴结活检而不是骨髓活检。我们能够在无累积毒性的情况下控制患者的病情在4年的西妥昔单抗抗IL6治疗期间。免疫抑制性抗IL6治疗并未使COVID-19的两次发作恶化。
■一名16岁男性出现反复发烧，盗汗和其他一些非特异性症状。经过广泛的评估，切除淋巴结活检证实了iMCD-NOS的诊断。患者接受高剂量类固醇治疗，随后接受siltuximab治疗四年。该治疗具有良好的耐受性,仅轻度中性粒细胞减少症不导致剂量调整。在siltuximab上，患者出现2次轻度COVID-19发作.他对siltuximab的反应在四年中仍然有效。
■缺乏生物标志物或病原体鉴定提出了诊断挑战，需要淋巴结组织病理学来明确诊断iMCD。抗IL6（siltuximab）是推荐的一线治疗，抑制炎症和停止疾病进展。每3至6周静脉给药可影响患者的生活质量，促使对替代疗法的进一步研究。高剂量类固醇,利妥昔单抗,环孢菌素,他克莫司,来那度胺或联合化疗如利妥昔单抗-硼替佐米-地塞米松是根据疾病严重程度考虑的选择之一.
■总的来说，长期的siltuximab有效地控制了iMCD症状，并且该年轻人耐受性良好，他经历了两次轻微的COVID-19发作。
结论：诊断iMCD需要淋巴结活检而不是骨髓活检。我们能够在无累积毒性的情况下控制患者的病情在4年的西妥昔单抗抗IL6治疗期间。免疫抑制性抗IL6治疗并未使COVID-19的两次发作恶化。
