A retrospective, population-based study was conducted to evaluate the incidence, clinical features, and outcome of malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH) in adults. Between January 1996 and December 2009, eight out of 887 (0.9%) patients diagnosed with hematological malignancies developed aggressive M-HLH in an area inhabited by approximately 160,000 people. Thus the estimated annual incidence of M-HLH in adulthood was 0.36/100,000 individuals/year. The clinical course of M-HLH was aggressive in all patients. Six patients were treated with a modified HLH-94 protocol; three achieved remission (durable in one case) while the others did not respond and died within an average of 2.4 months (range 1.5-3.5) after M-HLH diagnosis. Infection complicating the course of M-HLH occurred in four (50%) patients, all of whom developed fulminant M-HLH and died. Although the small study population limits the results, the long observation period strengthens its value.

Acquired or secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening hyperinflammation syndrome caused mostly by various infectious agents, autoimmune disorders or malignancy. So far, only anecdotal cases of sHLH associated with multiple myeloma have been published. We provide a review of all these reports and include a previously not published case of myeloma-associated sHLH in a 59-year-old male with complex partial epilepsy. Due to aggressive course of sHLH, increased awareness is indicated in all patients with malignancies which develop unremitting fever, cytopenia and splenomegaly. Early diagnosis and immediate introduction of adequate therapy is crucial for the outcome of HLH.

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