EXPERT HELP DIAGNOSING AND MANAGING HLH 
Last Updated 1/26/23

HLH is a descriptive diagnosis: critical to recognize but equally critical to investigate and treat its many underlying contributors.

The optimal diagnosis and management of HLH depend on dozens of factors – and are constantly evolving with new tests, treatments, and discoveries. Every provider could use some help.

Herein we’ve collected the most recent consensus-based guidance manuscripts, organized by the HS and/or affiliated organizations and authored by HS experts, to assist with the diagnosis and management of HLH across many different contexts.

It is important to remember that these manuscripts reflect the state of the art at their writing. They should inform but not substitute for up-to-date, patient-specific, clinical judgement and providers are strongly encouraged to seek local, regional, or national guidance as needed.

Earliest Steps

• Shakoory, B. et al. The 2022 EULAR and ACR Points to Consider for the Early Diagnosis and Management of Suspected HLH/MAS (coming soon)

HLH Diagnosis

• Jordan, M. B. et al. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer 66, e27929, doi:10.1002/pbc.27929 (2019).

MAS Diagnosis

• Ravelli, A. et al. 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis & rheumatology 68, 566-576, doi:10.1002/art.39332 (2016).
• Ravelli, A. et al. Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. RMD Open 2, e000161, doi:10.1136/rmdopen-2015-000161 (2016).

HLH in Adults

• La Rosee, P. et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood 133, 2465-2477, doi:10.1182/blood.2018894618 (2019).

HLH in the ICU

• Hines, M. R. et al. Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults. Crit Care Med, doi:10.1097/CCM.0000000000005361 (2021).

HLH Requiring HSCT

• Ehl, S. et al. Recommendations for the Use of Etoposide-Based Therapy and Bone Marrow Transplantation for the Treatment of HLH: Consensus Statements by the HLH Steering Committee of the Histiocyte Society. J Allergy Clin Immunol Pract 6, 1508-1517, doi:10.1016/j.jaip.2018.05.031 (2018).

Malignancy-Associated HLH

• Lehmberg, K. et al. Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies. Haematologica 100, 997-1004, doi:10.3324/haematol.2015.123562 (2015).
• Daver, N. et al. A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults. Cancer 123, 3229-3240, doi:10.1002/cncr.30826 (2017).