Castleman病(CD),也被称为血管滤泡增生,是一种罕见的疾病,其特征是由过量的白介素6(IL-6)分泌引起的非恶性纵隔淋巴结肿大。它可以是单中心或多中心(MCD)。这里,我们描述了一个有艾滋病病史的27岁男子,卡波西肉瘤(KS),以及因持续疲劳而出现在急诊室的潜伏梅毒,发烧,发冷,盗汗,和生产性咳嗽。传染病检查是阴性的,尽管经验性抗生素治疗,患者仍继续高烧。进行骨髓活检,恶性肿瘤阴性。患者最终接受了左锁骨淋巴结活检,显示浆细胞变异CD,对人类疱疹病毒8(HHV-8)具有阳性免疫染色,和高HHV-8病毒载量。我们开始给病人服用利妥昔单抗和脂质体阿霉素,但不幸的是,患者对利妥昔单抗有严重的过敏反应,所以我们不能继续这种治疗。我们,因此,开始托珠单抗治疗,改善了患者的一般状况,他最终出院了.在11个月后的随访中,胸部和腹部的重复CT扫描显示治疗反应接近完全,整个淋巴结肿大和肝脾肿大减少.CD患者中IL-6的过度产生与炎性细胞因子的产生有关,并在肿瘤血管生成中发挥作用,这使得它成为IL-6靶向治疗的潜力。CD的诊断,尤其是MCD,需要高度怀疑,淋巴结活检在诊断中至关重要。Tocilizumab,IL-6受体抗体,可能被认为是治疗对初始利妥昔单抗治疗不耐受或无反应的HHV-8阳性MCD患者的一种实用治疗方法.
Castleman disease (CD), also known as angiofollicular hyperplasia, is a rare disorder characterized by nonmalignant mediastinal lymph node enlargement provoked by excess interleukin-6 (IL-6) secretion. It could be unicentric or multicentric (MCD). Here, we describe a 27-year-old man with a prior history of AIDS, Kaposi sarcoma (KS), and latent syphilis who presented to the ED for persistent fatigue, fever, chills, night sweats, and productive cough. Infectious workup was negative, and the patient continued to have a high fever despite empiric antibiotic therapy. Bone marrow biopsy was performed and was negative for malignancy. The patient eventually underwent a left clavicular lymph node biopsy, which showed a plasma cell variant CD with positive immunostaining for human herpesvirus 8 (HHV-8), and high HHV-8 viral load. We started the patient on rituximab and liposomal doxorubicin, but unfortunately, the patient had a severe anaphylactic reaction to the rituximab, so we could not proceed with this treatment. We, therefore, started tocilizumab treatment, which improved the patient\'s general condition, and he was eventually discharged from our hospital. Upon follow-up 11-months later, a repeat CT scan of the chest and abdomen showed a near-complete treatment response with decreased lymphadenopathy throughout and hepatosplenomegaly. IL-6 overproduction in patients with CD is linked to the production of inflammatory cytokines and has a role in tumor angiogenesis, which makes it potential for IL-6 targeted therapy. The diagnosis of CD, especially MCD, requires a high index of suspicion, and a lymph node biopsy is essential in the diagnosis. Tocilizumab, an IL-6 receptor antibody, could potentially be considered as a practical therapeutic approach in managing HHV-8 positive MCD patients who do not tolerate or respond to initial rituximab therapy.