Table 1.

Patient characteristics

Patient	Age (years)1/Sex	Diagnosis2	Histology	Monotypia by flow cytometry / monoclonality of B-cells	Treatment	OS (years)3	Tissue4	Genomics5	TMB (mut/mb)	cfDNA6	Comment
1	29/F	iMCD	Hyaline vascular	Polytypic / ND	Siltuximab Sirolimus Anakinra	2.7+	LN	No alterations	3	No alterations
2	30/F	iMCD	Plasma cell	ND	Siltuximab Rituximab + chemotherapy Rituximab monotherapy	4.5+	LN	No alterations	N/A	No alterations
3	58/F	iMCD	Plasma cell	Polytypic / polyclonal	Siltuximab	1.9+	LN	NGS: 14q32–1p35 rearrangement Karyotype: der(1)dup(1)(q42q21)del(1)(q42)	N/A	NF1 K2459fs (0.3%)	IL-6 R resides on 1q21
4	40/M	iMCD	Plasma cell	ND	Observation	6.5+	Skin	NGS: KDM5C Q836	N/A	No alterations
5	40/F	UCD	Hyaline vascular	Polytypic / ND	Resection	3.3+	LN	NGS: TNS3-ALK fusion	1	ND	TNS3-ALK fusion would be predicted to inactivate ALK

Abbreviations: ALK: Anaplastic lymphoma kinase; cfDNA: cell free DNA; F: female; fs: frame shift; iMCD: idiopathic multicentric Castleman disease; ND: not done; LN: lymph node; M: male; mb: megabase pair; mut: mutation; N/A: not available; ND: not done; NF1: neurofibromin 1; NGS: next generation sequencing; OS: overall survival; TNS3: Tensin3; TMB: tumor mutation burden; UCD: unicentric Castleman disease

¹Age at diagnosis

²Cases of iMCD had to meet the diagnostic criteria outlined by D. Fajgenbaum et al.³¹

³From diagnosis

⁴Tissue used for next generation sequencing.

⁵Patients 1,2,4, and 5 underwent sequencing using the Foundation One Heme panel. Patient 3 underwent sequencing using the University of California San Diego Comprehensive NGS mutation panel analysis.

⁶Patients 1,2,3, and 4 underwent cfDNA analysis using the Guardant360 assay.