Christopher Cheung


Hemophagocytic lymphohistiocytosis (HLH) is rare in adults.  Two exemplary cases are presented here illustrating the clinical features, treatment and natural history of this aggressive and often fatal disease.  A 21 year old man presented with  jaundice and fever and was found to have CMV-triggered HLH.  He responded well to the HLH-2004 protocol and remains in remission.  A 45 year old woman presented with erythroderma and multi-organ failure and was found to have HLH associated with an abnormal T-cell population and low-titre EBV.  She died despite etoposide-based therapy.  HLH should be considered in patients with persistent unexplained inflammation, cytopenias, hepatitis, and coagulopathy.  Serum ferritin is a useful screening test and hyperferritinemia > 10 000 μg/L is thought to be specific for hemophagocytosis.  Tissue biopsy (bone marrow, lymph node or liver) to confirm hemophagocytosis is desirable, but is often a late finding and is not necessary for the diagnosis of HLH.  Once the diagnosis is confirmed, rapid initiation of chemo-immunotherapy is required to induce remission.

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