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
07/25/2021Pubmed ID
34302703DOI
10.1002/pbc.29261Scopus ID
2-s2.0-85111377456 (requires institutional sign-in at Scopus site) 9 CitationsAbstract
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 JAuthor
Allison S. Remiker MD Assistant Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdolescentAntibodies, Monoclonal
Castleman Disease
Female
Humans
Interleukin-6
Janus Kinases
Male
Nitriles
Pyrazoles
Pyrimidines