Rheumatology

HLH

Questions discussed in this category

How would you approach escalation of therapy in an adult patient with refractory Still’s disease and associated MAS/HLH (ferritin >100,000, transaminitis, DIC) despite high-dose steroids, high-dose anakinra, tocilizumab, and ruxolitinib?

Subsequently, emapalumab was initiated with a loading dose, followed by etoposide, which led to improvement in both symptoms and laboratory values. I...

1 Answer available

How do you approach the initial treatment for a patient presenting with MAS/HLH as the initial manifestation of SLE?

Patient presenting with seizure, rash, abnormal brain MRI, inflammatory CSF, ferritin >20k, leukocytosis, abnormal LFTs, positive ANA, positive Smi...

2 Answers available

When would you consider use of emapalumab for HLH/MAS ?

1 Answer available

What approaches can we take to initiate therapy and improve survival rates in patients with HLH?

2 Answers available

How do you utilize cytokine panels in your clinical practice?

Is there utility when classic inflammatory markers (ESR,CRP) or disease specific markers (C3, C4, dsDNA) do not correlate with patients disease activi...

1 Answer available

Should testing for genetic causes of HLH be performed in all patients with MAS or secondary HLH regardless of the patient's age?

2 Answers available

How do you approach dosing of anakinra in MAS?

1 Answer available

How do you approach diagnosis and treatment of HLH/MAS following CAR T-cell therapy?

How is this entity distinct from other secondary HLH entities?

2 Answers available

What is your approach to treatment of infection-triggered HLH that does not respond to treatment of the underlying infection?

2 Answers available

In the setting of secondary HLH associated with initial diagnosis of lymphoma, would you adjust steroid dose and type to account for differences in HLH and lymphoma treatment protocols?

1 Answer available