TAFRO (thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin myelofibrosis, renal dysfunction, and organomegaly) clinical subtype of idiopathic multicentric Castleman disease (iMCD-TAFRO) is a rare lymphoproliferative disease characterized by systemic inflammation. First-line treatment for iMCD-TAFRO includes steroids and interleukin (IL)-6 blockade. Many patients have refractory disease, which is associated with significant morbidity and mortality, and treatment remains challenging. We present two pediatric cases of iMCD-TAFRO. One patient responded to IL-6 blockade; the other was refractory to siltuximab and chemotherapy, ultimately responding to JAK inhibition with ruxolitinib. This is the first reported pediatric case of refractory iMCD-TAFRO responding to JAK inhibition.

中文翻译：

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TAFRO 临床亚型对 IL-6/JAK 抑制有反应的特发性多中心 Castleman 病：儿科病例系列

TAFRO（血小板减少症、全身性水肿、发热/C反应蛋白升高、网状纤维化、肾功能不全和器官肿大）是特发性多中心Castleman病（iMCD-TAFRO）的临床亚型，是一种罕见的以全身炎症为特征的淋巴组织增生性疾病。iMCD-TAFRO 的一线治疗包括类固醇和白细胞介素 (IL)-6 阻断。许多患者患有难治性疾病，这与显着的发病率和死亡率相关，治疗仍然具有挑战性。我们介绍了两个 iMCD-TAFRO 儿科病例。一名患者对 IL-6 阻断有反应；另一个对 siltuximab 和化疗无效，最终对 ruxolitinib 的 JAK 抑制有反应。这是首例报道的对 JAK 抑制有反应的难治性 iMCD-TAFRO 儿科病例。