Table 2.

Published clinical studies in RS.

Ref	Drugs	Study features	Number of patients	Schedule	CR (%)	ORR (%)	OS (months)	PFS (months)	Grade 3/4 adverse events
(102)	Pembrolizumab	Phase II unicentric clinical trial	25, including: - Relapsed CLL: 16 - RS: 9	200 mg/ 3 weeks	11	44	10.7	5.4	Thrombocytopenia (20%) Anemia (20%) Neutropenia (20%) dyspnea (8%) Hypoxia (8%)
(128)	Pembrolizumab or Nivolumab	Single center experience	10 RS: Nivolumab: 7 Pembrolizumab: 3	Doses according to their US label	10	UD	4.2	1.2	UD
(129)	Nivolumab and Ibrutinib	Phase II clinical trial	13, including: - Relapsed/ refractory CLL: 5 - CLL treated with ibrutinib for ≥ 9 months with persistent disease: 3 - RS: 5	Nivolumab 3mg/kg/15 weeks (cycle 1) Nivolumab 3mg/kg/15 weeks + Ibrutinib 420mg/day (cycle 2)	60	UD	UD	UD	/
(103)	Nivolumab and Ibrutinib	Phase 1/2a open-label multicentric clinical trial	141, including: - CLL/SLL: 36 - de novo DLBCL: 45 - FL: 40 - RS: 20	Nivolumab 3mg/kg / 15weeks + Ibrutinib 420 or 560 mg/day.	10	65	10.3	5	Neutropenia (25%), Anemia (35%), Thrombocytopenia (10%) Pneumoniae (10%) Rash (10%) Dyspnea (10%) hypotension (15%)

CLL, chronic lymphocytic leukemia; CR, complete response; DLBCL, diffuse large B-cell lymphoma; FL, follicular lymphoma; ORR, overall response rate; OS, overall survival; PFS, progression-free survival; SLL, small lymphocytic lymphoma; RS, Richter syndrome; UD, undetermined.