EM1709E "A Multidisciplinary Approach to a Complex Clinical Syndrome: HLH-MAS Task Force Presentation" (IM GR-090817)
Hemophagocytic lymphohistiocytosis (HLH) and Macrophage activation syndrome (MAS) are complex hyperinflammatory syndromes, which are difficult to distinguish from issues such as sepsis, as the root cause (high level proinflammatory cytokine production) is very similar. This causes confusion among treating physicians, and diagnostic/treatment delays. Furthermore, in adult patients, HLH is often associated with hematolymphoid malignancies, and a thorough search for these should be undertaken in patients in whom HLH is suspected or diagnosed, so as to best direct treatment. The treatment of HLH in children (in whom genetic defects in T cell function often underlie the disease) is standardized and protocol driven. In adults it is currently quite variable, in part due to the variability in underlying causes. The delays in diagnosis, the frequent associations with malignancy and the variability in treatment approaches, all contribute to poorer mortality in adult HLH patients. Recognizing the need for a consensus, multidisciplinary approach to these complicated patients, and the need for research to better their care, we have created a multidisciplinary working group, which is accessible to all physicians at UT Southwestern and Parkland, to facilitate this.
UT Southwestern faculty, fellows, residents and medical students, community physicians, nurse clinicians, physician assistants and nurses.
At the conclusion of this activity, the participant should be able to:
- Understand the pathophysiology of Hemophagocytic lymphohistiocytosis (HLH) and Macrophage activation syndrome (MAS).
- Understand the clinical presentation of HLH/MAS.
- Understand the diagnostic criteria and lab evaluation for HLH and Macrophage activation syndrome.
- Understand the illnesses which can predispose to HLH in adults, such that evaluation for these is not delayed.
- Understand how to contact/mobilize the HLH/MAS task force to aid in the evaluation and treatment of these patients, and advance the study of them.
Christian A. Wysocki, M.D., Ph.D.
Division of Allergy and Immunology
Chair of Adult HLH/MAS Task Force
Panelists: Corey Kershaw, MD; Srikanth Nagalla, MD; Shannan Tujios, MD; Bonnie Prokesch, MD; Weina Chen, MD, PhD; Fatemeh Ezzati, MD; and Arturo Dominguez, MD
- 1.00 AMA