Storming the Castle: A Case Report of Multi-system Dysregulation in a Child with Castleman Disease

Overview

Journal Clin Case Rep

Date 2022 Apr 4

PMID 35369387

Authors

Samantha J DeMarsh

Nicole E Kendel

Christine Bacha

Stacy P Ardoin

Samir Kahwash

Melissa J Rose

Affiliations

Soon will be listed here.

Abstract

Castleman disease is a non-clonal, lymphoproliferative disorder rarely seen in children. Presented is a 12-year-old male with progressive abdominal pain, vomiting, and fever. Diagnostic testing revealed multi-organ system involvement and the diagnosis was ultimately made with tissue biopsy. Marked disease regression occurred after high-dose steroids and continued interleukin-6 inhibition.

Citing Articles

Storming the castle: A case report of multi-system dysregulation in a child with Castleman disease.

DeMarsh S, Kendel N, Bacha C, Ardoin S, Kahwash S, Rose M Clin Case Rep. 2022; 10(2):e05491.

PMID: 35369387 PMC: 8858787. DOI: 10.1002/ccr3.5491.

References

Fajgenbaum D, Uldrick T, Bagg A, Frank D, Wu D, Srkalovic G . International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017; 129(12):1646-1657. PMC: 5364342. DOI: 10.1182/blood-2016-10-746933. View

Chaturvedi S, Siegel D, Wagner C, Park J, van de Velde H, Vermeulen J . Development and validation of panoptic Meso scale discovery assay to quantify total systemic interleukin-6. Br J Clin Pharmacol. 2015; 80(4):687-97. PMC: 4594705. DOI: 10.1111/bcp.12652. View

Nishimoto N, Kanakura Y, Aozasa K, Johkoh T, Nakamura M, Nakano S . Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease. Blood. 2005; 106(8):2627-32. DOI: 10.1182/blood-2004-12-4602. View

Jordan M, Allen C, Greenberg J, Henry M, Hermiston M, Kumar A . Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer. 2019; 66(11):e27929. PMC: 7340087. DOI: 10.1002/pbc.27929. View

Fajgenbaum D . Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Blood. 2018; 132(22):2323-2330. PMC: 6265649. DOI: 10.1182/blood-2018-05-848671. View

van Rhee F, Voorhees P, Dispenzieri A, Fossa A, Srkalovic G, Ide M . International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. 2018; 132(20):2115-2124. PMC: 6238190. DOI: 10.1182/blood-2018-07-862334. View

DOsualdo A, Picco P, Caroli F, Gattorno M, Giacchino R, Fortini P . MVK mutations and associated clinical features in Italian patients affected with autoinflammatory disorders and recurrent fever. Eur J Hum Genet. 2004; 13(3):314-20. DOI: 10.1038/sj.ejhg.5201323. View

Iwaki N, Fajgenbaum D, Nabel C, Gion Y, Kondo E, Kawano M . Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease. Am J Hematol. 2016; 91(2):220-6. DOI: 10.1002/ajh.24242. View

Parez N, Bader-Meunier B, Roy C, Dommergues J . Paediatric Castleman disease: report of seven cases and review of the literature. Eur J Pediatr. 1999; 158(8):631-7. DOI: 10.1007/s004310051166. View

10.

Farruggia P, Trizzino A, Scibetta N, Cecchetto G, Guerrieri P, DAmore E . Castleman's disease in childhood: report of three cases and review of the literature. Ital J Pediatr. 2011; 37:50. PMC: 3219574. DOI: 10.1186/1824-7288-37-50. View

11.

DeMarsh S, Kendel N, Bacha C, Ardoin S, Kahwash S, Rose M . Storming the castle: A case report of multi-system dysregulation in a child with Castleman disease. Clin Case Rep. 2022; 10(2):e05491. PMC: 8858787. DOI: 10.1002/ccr3.5491. View

12.

Cervantes C, Correa R . Castleman Disease: A Rare Condition with Endocrine Manifestations. Cureus. 2016; 7(11):e380. PMC: 4689570. DOI: 10.7759/cureus.380. View

13.

Cuisset L, Drenth J, Simon A, Vincent M, van der Velde Visser S, van der Meer J . Molecular analysis of MVK mutations and enzymatic activity in hyper-IgD and periodic fever syndrome. Eur J Hum Genet. 2001; 9(4):260-6. DOI: 10.1038/sj.ejhg.5200614. View

14.

Yoshizaki K, Murayama S, Ito H, Koga T . The Role of Interleukin-6 in Castleman Disease. Hematol Oncol Clin North Am. 2017; 32(1):23-36. DOI: 10.1016/j.hoc.2017.09.003. View