Sánchez-Pardo Santiago, Rojas-Holguín Daniel Felipe, Choi-Park Isabela, Osorio-Ramírez Jaime Andrés, Bolívar-Mejía Adrián
Universidad Industrial de Santander, Facultad de Medicina, Departamento de Medicina Interna. Bucaramanga, Colombia.
Rev Med Inst Mex Seguro Soc. 2020 Nov 4;58(6):734-739. doi: 10.24875/RMIMSS.M20000107.
Multicentric Castleman’s disease is a rare benign lymphoproliferative disorder that involves multiple enlarged lymph nodes in different areas of the body. Recently, a unique clinic pathological variant of this disease called TAFRO syndrome has been described.
A 23-year-old male with a history of one year and six months of evolution characterized by generalized lymphadenopathy with, night diaphoresis, dyspnea and weight loss. At the third month, hypertension was diagnosed and 8 months later type 2 diabetes mellitus. After his admission, microcytic anemia, thrombocytopenia, renal dysfunction, pleural effusion and ascites were documented. Based on the findings of the physical examination, a computed tomography scan was performed, where multiple lymph nodes and hepatosplenomegaly were identified. A lymph node biopsy was performed, which reported Castleman’s disease, considering then a diagnosis of TAFRO syndrome due to its association with thrombocytopenia, microcytic anemia, anasarca, fever, renal dysfunction, adenomegaly and hepatosplenomegaly.
There are still doubts about whether it corresponds to a different disease or a subtype of the Castleman disease. It is more frequent in women, occurs in the middle-aged and elderly and given its heterogeneous presentation, it usually represents a diagnostic challenge. Although its management is not standardized, therapeutic options include immunosuppressant such as steroids, cyclosporine, rituximab and anti-IL6.
多中心Castleman病是一种罕见的良性淋巴增殖性疾病,累及身体不同部位的多个肿大淋巴结。最近,已描述了该疾病的一种独特临床病理变异型,称为TAFRO综合征。
一名23岁男性,病程1年6个月,表现为全身淋巴结肿大、夜间盗汗、呼吸困难和体重减轻。第三个月诊断为高血压,8个月后诊断为2型糖尿病。入院后,记录到小细胞贫血、血小板减少、肾功能不全、胸腔积液和腹水。根据体格检查结果,进行了计算机断层扫描,发现多个淋巴结及肝脾肿大。进行了淋巴结活检,报告为Castleman病,鉴于其与血小板减少、小细胞贫血、全身水肿、发热、肾功能不全、腺肿大及肝脾肿大相关,随后诊断为TAFRO综合征。
对于它是一种不同的疾病还是Castleman病的一个亚型仍存在疑问。它在女性中更常见,发生于中老年人,鉴于其表现多样,通常构成诊断挑战。尽管其治疗不规范,但治疗选择包括免疫抑制剂,如类固醇、环孢素、利妥昔单抗和抗IL6。