HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN ADULTS: TWO INSTRUCTIVE CASES

Abstract

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.