How do you approach management autoimmune lymphoproliferative syndrome (ALPS) complicated by HLH?

Excellent and tough question. There are no direct guidelines for this specific question. We will have to extrapolate from other evidence and clinical reasoning.

Investigation of the trigger must occur in parallel with management. I will discuss how I approach investigation first and then management.

I...

HLH may respond to Gamifant and Anakinra - monitor CXCL19 cytokine.