Table 2.

Use of published protocols in the management of sHLH/MAS post-HSCT or CAR-T cell therapy.

Published protocol	Components of protocol	Centres (N)
MD Anderson (post CAR-T cell) (44)	Supportive organ-specific treatment, broad-spectrum antibiotics, IV Tocilizumab or Siltuximab (anti-IL6 agents), IV corticosteroids	4
HLH-2004 (for fHLH) (24)	8 weeks initial therapy with IV dexamethasone and Etoposide. Then ciclosporin is introduced, dexamethasone continues to be pulsed and etoposide continued whilst awaiting a donor for BMT	2
La Rosee et al. (45)	Use of corticosteroids +/- IVIG in most cases with addition of etoposide (if malignancy-triggered), ciclosporin & anakinra (if autoimmune-related) or anti-IL-6 (if CAR-T cell related)	1
HLH-94 (for fHLH)	8 weeks initial therapy with IV dexamethasone and Etoposide before proceeding to definitive treatment with BMT	1