Department of Infectious Disease.
Libyan J Med. 2008 Jun 1;3(2):101-3. doi: 10.4176/080108.
Castleman's Disease (CD) is a rare lymphoproliferative disorder accompanied by marked systemic inflammatory response. Morphological diagnosis of CD requires biopsy of the whole of the involved lymph node tissue. Three histologic variants have already been described in CD morphology (hyaline vascular, plasma-cell, and mixed). In this study, we report a case of a multicentric Castleman's disease of the plasma cell variant type with negative Herpes Virus 8. The clinical presentation of this patient was of systemic amyloidosis as a result of both a delayed diagnosis and medical management. Previously described cases of CD with secondary amyloidosis have been of the localized type. Regardless, long-standing clinical remission of CD by cytotoxic drugs and anti-CD20 antibody therapy was achieved, but the nephrotic syndrome remained irreversible.
卡斯尔曼病(CD)是一种罕见的淋巴组织增生性疾病,伴有明显的全身炎症反应。CD 的形态学诊断需要对整个受累淋巴结组织进行活检。CD 的形态学已经描述了三种组织学变异(透明血管型、浆细胞型和混合型)。在本研究中,我们报告了一例浆细胞变异型多中心卡斯尔曼病,疱疹病毒 8 阴性。该患者的临床表现为系统性淀粉样变性,这是由于诊断和医疗管理的延误。以前描述的伴有继发性淀粉样变性的 CD 病例为局限性类型。然而,通过细胞毒性药物和抗 CD20 抗体治疗实现了 CD 的长期临床缓解,但肾病综合征仍然不可逆转。