Pediatric TAFRO syndrome: A multi‐institution case series illustrating clinical challenges and excellent outcomes

Author:

Johnson Amanda K.1,Goteti Sasidhar1ORCID,Devald Bar1,Mestnik Shelby2,Ghosh Taumoha1,Williams Robin2,Doughty Elizabeth S.3,Linden Michael A.4,Beckman Amy4ORCID,Williams Todd5,Richardson Kelsey6,Martinelli Marie7

Affiliation:

1. Division of Pediatric Hematology/Oncology University of Utah/Intermountain Health Primary Children's Hospital Salt Lake City Utah USA

2. Division of Pediatric Hematology/Oncology University of Minnesota/M Health Fairview Masonic Children's Hospital Minneapolis Minnesota USA

3. Department of Pathology University of Utah Salt Lake City Utah USA

4. Department of Pathology University of Minnesota/M Health Fairview Masonic Children‘s Hospital Minnesota Minneapolis USA

5. Department of Pathology Oregon Health & Science University/Doernbecher Children‘s Hospital Oregon Portland USA

6. Division of Pediatric Nephrology Oregon Health & Science University/Doernbecher Children’s Hospital Portland Oregon USA

7. Division of Pediatric Hematology/Oncology Oregon Health & Science University/Doernbecher Children’s Hospital Portland Oregon USA

Abstract

AbstractA 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.

Publisher

Wiley

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