Treatment

How is hemophagocytic lymphohistiocytosis (HLH) treated?

Chemotherapy

Etoposide, as outlined in the HLH-94 protocol, is commonly utilized in HLH. Additionally, if malignancy is identified as a triggering conditioning, specific chemotherapy regimens should be utilized.

Allogeneic stem cell transplant

Allogeneic HSCT is currently the only option for long-term cure in primary/familial HLH.

Plasma exchange

Rituximab

EBV-triggered HLH is treated with rituximab because EBV replicates in B cells.

Corticosteroids

High dose corticosteroids, commonly dexamethasone, are used in HLH to deplete T lymphocytes.

The patient is started on dexamethasone with rapid improvement in his clinical symptoms as well as laboratory indices. He is discharged with plan for a prolonged corticosteroid taper.

At outpatient follow-up 1 month after discharge, he reports no recurrence of his presenting symptoms. Outpatient lab work demonstrates complete normalization of his inflammatory works and cytopenias.

His final diagnosis is acquired hemophagocytic lymphohistiocytosis due to probable undefined infection.

What is the overall mortality of secondary hemophagocytic lymphohistiocytosis (HLH)?

10%

20%

30%

40%

50%

As suggested by published case series. Patients with malignancy associated HLH appear to suffer a worse prognosis.

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