Pediatric Hematology/Oncology

Pediatric Rheumatology

Questions discussed in this category

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

1 Answer available

How would you treat suspected secondary HLH in patients whom etoposide is contraindicated?

No clear inciting etiology found. Would you consider dexamethasone +/- cytokine blockade (IL-1, IL-6, or IFN-g)? What do you think about the data for...

1 Answer 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

What are the best labs to trend improvement in HLH?

1 Answer available

Would you discontinue anticoagulation in patients with antiphospholipid antibody syndrome, who have a remote history of thrombotic events and are now negative for pathogenic antiphospholipid antibodies?

Would the answer differ if the index event was arterial vs venous?

3 Answers available

Do you recommend the use of dexamethasone and anakinra as upfront treatment in a pediatric patient with secondary HLH (nonrheumatic) who is too ill to tolerate etoposide?

This type of etoposide sparing therapy has been previously described in a case series at https://pubmed.ncbi.nlm.nih.gov/32725881/

1 Answer available

Do you repeat antiphospholipid antibody testing in a patient that previously met criteria for APLS?

If so, how long after diagnosis do you do so?

1 Answer available

Papers discussed in this category

Arthritis Rheumatol, 2019 Dec 26

Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis.

Autoimmun Rev, 2017 Sep 09

Can we withdraw anticoagulation in patients with antiphospholipid syndrome after seroconvertion?

Pediatric blood & cancer, 2011-01

Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortality.

Ther Adv Musculoskelet Dis, 2020 Nov 24

Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still's disease: when dosage matters in overcoming secondary therapy resistance.

Blood, 2022 Jun 16

A study of ruxolitinib response-based stratified treatment for pediatric hemophagocytic lymphohistiocytosis.

Annals of the rheumatic diseases, 2023 Mar 31

Efficacy and safety of emapalumab in macrophage activation syndrome.

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