Hemophagocytic lymphohistiocytosis in Texas: Observations on ethnicity and race

BACKGROUND: Early recognition and aggressive treatment of hemophagocytic lymphohistiocytosis (HLH) has changed a uniformly fatal disease to one 55% survive. We examined the diagnosis and treatment of pediatric patients with HLH from the three largest academic medical centers in Texas for information on modern non-study treatment and survival. In contrast with previously reported series, the racial and ethnic composition of Texas provided a unique opportunity to evaluate the impact of race and ethnicity on survival with HLH. PROCEDURE: A retrospective chart review of local oncology and pathology databases identified 70 patients with HLH from 1992 to 2007. Median age was 1.8 years (range 0.1-16.5 years) and 43% were Latino. RESULTS: We identified 70 patients with an overall survival of 67% after a median follow-up of 3 months (range 1-139 months). Twenty patients (29%) underwent stem cell transplant (SCT). Seven patients (18%) had mutations in the Perforin, Munc 13-4, or Syntaxin-11 genes, consistent with primary disease. Calculated cross-sectional prevalence of HLH in Texas from our study is 1 in 100,000 children. The effect of Latino ethnicity on survival was not statistically significant. CONCLUSION: HLH is a rare but potentially treatable illness with modern aggressive therapy. Though treatment is more standardized for HLH, the role of race and ethnicity as risk factors for development of disease and impact on outcome may warrant further investigation.