Faculty Collaboration Database

Idiopathic multicentric Castleman disease with TAFRO clinical subtype responsive to IL-6/JAK inhibition: A pediatric case series. Pediatr Blood Cancer 2021 Oct;68(10):e29261

Date

07/25/2021

Pubmed ID

34302703

DOI

10.1002/pbc.29261

Scopus ID

2-s2.0-85111377456 (requires institutional sign-in at Scopus site)   9 Citations

Abstract

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.

Author List

Lust H, Gong S, Remiker A, Rossoff J

Author

Allison S. Remiker MD Assistant Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Antibodies, Monoclonal
Castleman Disease
Female
Humans
Interleukin-6
Janus Kinases
Male
Nitriles
Pyrazoles
Pyrimidines