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

Can a patient still have primary HLH even in the absence of any HLH associated genetic mutations?

rWGS did not demonstrate any HLH definable or associated mutations.

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

How would you adjust the steroid dose for steroid-induced psychosis in a patient being treated for secondary HLH with the HLH-94 protocol?

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

What is the difference between Multisystem Inflammatory Syndrome in Adults (MIS-A) and MAS/HLH?

1 Answer available

How do you approach relapsed idiopathic HLH?

2 Answers available

In what circumstances would you give G-CSF to a patient with severe neutropenia and HLH?

1 Answer available

Papers discussed in this category

Blood, 2019-06-06

Recommendations for the management of hemophagocytic lymphohistiocytosis in adults.

Eur J Haematol, 2021 Feb 17

Ruxolitinib as adjunctive therapy for secondary hemophagocytic lymphohistiocytosis: A case series.

ACR Open Rheumatol, 2021 Aug 25

Performance of Cytokine Storm Syndrome Scoring Systems in Pediatric COVID-19 and Multisystem Inflammatory Syndrome in Children.

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.

Frontiers in immunology, 2023 Jun 22

Approaching hemophagocytic lymphohistiocytosis.

Arthritis & rheumatology (Hoboken, N.J.), 2023 Jul 24

The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS).

Arthritis Rheum,

Low blood concentration of hydroxychloroquine is a marker for and predictor of disease exacerbations in patients with systemic lupus erythematosus.

Arthritis Rheumatol, 2020 Jan 07

Hydroxychloroquine Blood Levels Predict Hydroxychloroquine Retinopathy.

Annals of the rheumatic diseases, 2023 Oct 12

EULAR recommendations for the management of systemic lupus erythematosus: 2023 update.

Journal of clinical medicine, 2020 Aug 21

Glucocorticoids in . Ten Questions and Some Issues.

Lancet Rheumatol, 2025 Jul

ERN ReCONNET-SLICC-SLEuro expert consensus on the therapeutic management of rare systemic lupus erythematosus manifestations.

Arthritis care & research, 2025 Nov 03

2025 American College of Rheumatology (ACR) Guideline for the Treatment of Systemic Lupus Erythematosus.

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