Plasma Exchange Not a Promising Monotherapy in MAS-HLH

Plasma exchange should be used as a supportive measure, not a monotherapy for adult patients with MAS-HLH, according to a report published in the journal Lupus.

In this retrospective series of steroid refractory haemophagocytic lymphohistiocytosis (HLH; also referred to as macrophage activation syndrome (MAS) or more recently MAS-HLH), a total of 8 patients were treated with plasma exchange, inducing a partial response in 5 of 8 patients. An escalation of immunosuppressive therapies was required in all patients, and 6 out of 8 steroid refractory patients survived (median follow-up: 54.4 weeks). Relapses occurred in 2/8 cases.

One patients with new-onset adult-onset Still’s disease was escalated to etoposide but most patients with systemic lupus erythematosus-associated MAS-HLH responded well to cyclophosphamide.

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Reference

Lorenz G, Schul L, Schraml F, et al Adult macrophage activation syndrome-haemophagocytic lymphohistiocytosis: ‘of plasma exchange and immunosuppressive escalation strategies’ – a single centre reflection. Lupus. 2020. [Online ahead of print]. DOI: 10.1177/0961203320901594