Description:

Adult hematologists and internists have observed that the incidence of Hemophagocytic Lymphohistiocytosis (HLH) in adult patients appears to be increasing. Whether this represents a true increase in the occurrence of the syndrome or a reflection of ascertainment bias remains uncertain. In any case, since the vast majority of the literature on HLH is based on observations of the pediatric population, there is an increasing focus on the significant differences in presentation, natural history, and appropriate intervention between adult and pediatric patients with HLH. In this session, we will provide an overview of HLH and try to provide a context for approaching the disease as it presents in the adult population.

In the first talk, Dr. Allen will discuss the shared aspects of the pathophysiology of HLH and how the epidemiology of the disease diverges in children and adults, with emphasis on the overlap between familial and acquired HLH.

Dr. Berliner will focus on how the diagnostic criteria for HLH based on pediatric disease should be interpreted and perhaps modified to improve the diagnosis of HLH in the adult population.

In the third talk, Dr. La Rosée will discuss the therapeutic approach to adult HLH, including criteria for treatment, evaluation of treatment response, and the role of stem cell transplantation in the adult age group.