Reactive hemophagocytic syndrome--a clinicopathologic study of 40 patients in an Oriental population

Abstract

Purpose: The purpose of this investigation was to assess the clinical spectrum and outcome of reactive hemophagocytic syndrome (RHS) in an Oriental population.

Patients and methods: The investigation was a retrospective study of 40 consecutive patients with RHS diagnosed over a 4-year period in Queen Elizabeth Hospital, a community-based hospital in Hong Kong.

Results: The incidence of RHS in Hong Kong appeared to be comparable with that of Western countries. However, the spectrum of diseases associated with RHS was distinctly different. Forty percent of the cases were associated with malignant lymphoma. Another 40% of the cases were associated with infection, often due to bacteria, and infection due to the herpes group of viruses was uncommon, probably reflecting ethnic and geographic differences from the white population. Two patients had the acute lupus hemophagocytic syndrome, a distinctive form of RHS that occurs in patients with systemic lupus erythematosus. Treatment was basically supportive, with specific therapy also given for the underlying disease if present. Eighteen patients (45%) died of the acute disease, mostly as a result of complications of RHS or the underlying diseases. The development of disseminated intravascular coagulopathy was a poor prognostic factor.

Conclusions: RHS is an uncommon disorder in Hong Kong and is often associated with malignant lymphoma or bacterial infection. A rigorous search for an underlying malignancy or infection is thus warranted once this diagnosis is made.