Division of Pediatric Hematology/Oncology, University of Utah/Intermountain Health Primary Children's Hospital, Salt Lake City, Utah, USA.
Division of Pediatric Hematology/Oncology, University of Minnesota/M Health Fairview Masonic Children's Hospital, Minneapolis, Minnesota, USA.
Pediatr Blood Cancer. 2024 Oct;71(10):e31234. doi: 10.1002/pbc.31234. Epub 2024 Jul 31.
A rare lymphoproliferative disorder involving thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), renal dysfunction (R), and organomegaly (O), called TAFRO syndrome, was first reported in 2010. Considered a variant of idiopathic multicentric Castleman's disease, the recent discovery and rarity of this syndrome pose challenges to diagnosis and management. Herein, we review three pediatric cases, including an infant, that illustrate the heterogeneity of TAFRO syndrome. Despite differences in presentation and treatment responses, all patients experienced excellent outcomes. This multi-institutional case series highlights the need to work toward earlier diagnosis and improved long-term management recommendations for patients with TAFRO syndrome.
TAFRO 综合征是一种罕见的涉及血小板减少症(T)、全身性水肿(A)、发热(F)、网状纤维纤维化(R)、肾功能障碍(R)和器官肿大(O)的淋巴组织增生性疾病,于 2010 年首次报道。该综合征被认为是特发性多中心 Castleman 病的一种变体,由于其最近才被发现且较为罕见,因此给诊断和治疗带来了挑战。在此,我们回顾了三例儿科病例,包括一例婴儿病例,这些病例说明了 TAFRO 综合征的异质性。尽管临床表现和治疗反应存在差异,但所有患者均获得了极好的结果。该多机构病例系列强调了需要努力实现早期诊断,并为 TAFRO 综合征患者制定更好的长期管理建议。