关键词: Castleman's disease anemia fever lymphoproliferative disorders organomegaly (TAFRO) syndrome renal dysfunction reticulin fibrosis thrombocytopenia

Mesh : Adolescent Female Humans Infant Male Castleman Disease / pathology diagnosis therapy Edema / pathology etiology Fever / etiology Syndrome Thrombocytopenia / therapy diagnosis pathology

来  源:   DOI:10.1002/pbc.31234

Abstract:
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.
摘要:
一种罕见的淋巴细胞增殖性疾病,涉及血小板减少症(T),anasarca(A),发烧(F),网织蛋白纤维化(R),肾功能不全(R),和器官肿大(O),叫做TAFRO综合征,于2010年首次报道。被认为是特发性多中心Castleman病的一种变种,这种综合征的最新发现和罕见给诊断和治疗带来了挑战。在这里,我们回顾了三例儿科病例,包括一个婴儿,这说明了TAFRO综合征的异质性。尽管在演示和治疗反应方面存在差异,所有患者均获得了优异的结果.这个多机构病例系列强调了对TAFRO综合征患者进行早期诊断和改进长期管理建议的必要性。
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