Challenges of Diagnosing HLH
A consensus review on how to improve diagnosis of familial HLH
Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO). Jordan MB, Allen CE, Greenberg J, et al. Pediatr Blood Cancer. 2019;66(11):e27929. doi:10.1002/pbc.27929
This consensus review provides a conceptual framework and two concepts to clarify HLH diagnosis and treatment: "HLH disease" and "HLH disease mimics." HLH subtypes are divided by the specific etiologic associations rather than the ambiguous dichotomy of "primary" and "secondary." Extensive expert-based advice regarding the diagnosis and initiation of treatment for patients with HLH, rooted in improved understanding of its pathophysiology, is provided.
The supplement to this review provides detailed and practical tools for diagnosing and treating HLH. They can be reached at the following link (under supporting information):