Archive | 2019
Genetics of Primary Hemophagocytic Lymphohistiocytosis
Abstract
In this chapter we describe the genes responsible for familial or primary hemophagocytic lymphohistiocytosis, and the clinical and laboratory features that are associated with each syndrome. Additionally, we place primary hemophagocytic lymphohistiocytosis into four distinct categories, depending on the presence or absence of hypopigmentation and susceptibility to Epstein–Barr virus-induced lymphoproliferative disease.