Nephrology Center, Toranomon Hospital, Tokyo, 105-0001, Japan.
Nephrology Center, Toranomon Hospital, Tokyo, 105-0001, Japan.
Hum Pathol. 2018 Dec;82:258-263. doi: 10.1016/j.humpath.2018.03.021. Epub 2018 Apr 4.
An 84-year-old Japanese man was admitted due to anasarca, thrombocytopenia, systemic inflammation, and progressive renal insufficiency, resistance to diuretics, glucocorticoid therapy, and plasma exchange. Renal biopsy showed diffuse endocapillary proliferation and mesangiolysis without any immune deposits. Tocilizumab suppressed systemic inflammation, resulting in improvement of anasarca and renal dysfunction, but thrombocytopenia persisted and platelet-associated IgG antibody was elevated. Although romiplostim was effective for thrombocytopenia, the patient died of aspiration pneumonia after cerebral hemorrhage. Autopsy showed hyaline vascular-type Castleman disease-like lymphadenopathy and reticulin myelofibrosis with an increase of megakaryocytes. Renal finding showed that endocapillary injury improved, and collapsed glomeruli were noted. This patient fitted the criteria for TAFRO (thrombocytopenia [T], anasarca [A], fever [F], reticulin myelofibrosis [R], and organomegaly [O]) syndrome. The clinical course suggests that 2 factors, including overproduction of interleukin 6 and autoimmune-mediated thrombocytopenia via thrombopoietin receptor, may have contributed to the pathogenesis of TAFRO syndrome in this patient.
一位 84 岁的日本男性因全身水肿、血小板减少、全身炎症和进行性肾功能不全、利尿剂抵抗、糖皮质激素治疗和血浆置换而入院。肾活检显示弥漫性毛细血管内增殖和系膜溶解，没有任何免疫沉积物。托珠单抗抑制全身炎症，导致全身水肿和肾功能不全改善，但血小板减少持续存在，血小板相关 IgG 抗体升高。虽然罗米司亭对血小板减少有效，但患者在脑出血后继发吸入性肺炎死亡。尸检显示透明血管型 Castleman 病样淋巴结病和网状纤维骨髓纤维化，伴有巨核细胞增多。肾脏发现内皮层损伤改善，可见塌陷的肾小球。该患者符合 TAFRO（血小板减少[T]、全身水肿[A]、发热[F]、网状纤维骨髓纤维化[R]和器官肿大[O]）综合征标准。临床病程提示，包括白细胞介素 6 过度产生和通过血小板生成素受体的自身免疫性血小板减少症在内的 2 个因素可能促成了该患者 TAFRO 综合征的发病机制。
