Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2021 Oct;68(10):e29261.
doi: 10.1002/pbc.29261. Epub 2021 Jul 24.

Idiopathic multicentric Castleman disease with TAFRO clinical subtype responsive to IL-6/JAK inhibition: A pediatric case series

Hannah Lust  1 , Shunyou Gong  2 , Allison Remiker  1   3 , Jenna Rossoff  1   3
Affiliations
Case Reports

Idiopathic multicentric Castleman disease with TAFRO clinical subtype responsive to IL-6/JAK inhibition: A pediatric case series

Hannah Lust et al. Pediatr Blood Cancer. 2021 Oct.

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.

Keywords: Castleman disease; JAK inhibition; idiopathic; ruxolitinib; siltuximab.

PubMed Disclaimer

References

REFERENCES

    1. Sopfe J, Endres A, Campbell K, et al. Castleman disease in pediatrics: insights on presentation, treatment, and outcomes from a two-site retrospective cohort study. Pediatr Blood Cancer. 2019;66(5):e27613.
    1. Borocco C, Ballot-Schmit C, Ackermann O, et al. The French paediatric cohort of Castleman disease: a retrospective report of 23 patients. Orphanet J Rare Dis. 2020;15(1):95.
    1. Van Rhee F, Oksenhendler E, Srkalovic G, et al. International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. Blood Adv. 2020;4(23):6039-6050.
    1. Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020;135(16):1353-1364.
    1. Masaki Y, Kawabata H, Takai K, et al. Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version. Int J Hematol. 2016;103(6):686-692.

Publication types

Supplementary concepts

LinkOut - more resources