ACR Meeting Abstracts

ACR Meeting Abstracts

  • Home
  • Meetings Archive
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018 ACR/ARHP Annual Meeting
    • 2017 ACR/ARHP Annual Meeting
    • 2017 ACR/ARHP PRSYM
    • 2016-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • Register
    • View and print all favorites
    • Clear all your favorites
  • Meeting Resource Center

Abstract Number: 1634

Etiologies and Management of Hemophagocytic Lymphohistiocytosis: Is It Time for an Updated Protocol and Targeted Treatments?

Therese Posas-Mendoza1, Cara McLeod1, William Davis2 and Robert Quinet2, 1Ochsner Medical Center, New Orleans, LA, 2Dept of Rheumatology, Ochsner Medical Center, New Orleans, LA

Meeting: ACR Convergence 2020

Keywords: autoimmune diseases, cytokines, Juvenile idiopathic arthritis, macrophage activation syndrome, Systemic lupus erythematosus (SLE)

  • Tweet
  • Email
  • Print
Save to PDF
Session Information

Date: Monday, November 9, 2020

Session Title: Miscellaneous Rheumatic & Inflammatory Diseases Poster III: Therapies

Session Type: Poster Session D

Session Time: 9:00AM-11:00AM

Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disease characterized by immune-overstimulation and a hyperinflammatory response resulting in cytokine storm and multi-organ failure.1 Secondary HLH usually occurs in adults in the setting of malignancy, infection, or a rheumatic condition. It is often triggered by an infection such as Epstein-Barr virus (EBV).1 Secondary HLH occurring in patients with autoimmune diseases is referred to as macrophage activation syndrome (MAS). The approach to the management of secondary HLH can be confusing and problematic due to the rarity of the condition and the heterogeneity of treatment approaches for HLH. The aim of this study was to study the patients diagnosed with hemophagocytic lymphohistiocytosis at our center, and to compare their predisposing conditions, treatments, and outcomes.

Methods: We conducted a retrospective chart review of all patients over 18 years of age diagnosed with hemophagocytic lymphohistiocytosis at our center between 2013 and 2019. Patients who met HLH-2004  or H-score criteria were included in this study. The patients’ presentations, management, and outcomes were analyzed. 

Results: Twenty-nine patients with hemophagocytic lymphohistiocytosis met inclusion criteria. 7 patients had a malignancy, 11 had a rheumatic disease, 6 had an infection alone, 2 were transplant patients, and 3 had a combination of malignancy, rheumatic disease, and/or infection. Of all the patients treated with the HLH-94 protocol 73% (11/15) died, 13% (2/15) had recurrence of HLH, and 6% (1/15) developed an invasive fungal infection. The patients with underlying rheumatologic conditions received targeted personalized therapy with pulse steroids, tocilizumab, anakinra, IVIg, cyclosporin, rituximab and/or cyclophosphamide in addition to antiviral/antibiotic therapy. All of the patients treated with targeted therapy had resolution of HLH.

Conclusion: Our findings suggest that the rheumatologic patient population responds well to a targeted and more personalized therapeutic approach, and poorly to the standard HLH-94 protocol. It is unclear whether the poor outcomes found with the use of the HLH protocol are secondary to the protocol itself, or the aggressive nature of malignancy-associated HLH. HLH is a rare life-threatening disease, and further studies are needed to develop more tailored therapeutic regimens.

Table 1: Therapeutic Approaches and Outcomes (1/2)

Table 1: Therapeutic Approaches and Outcomes (2/2)


Disclosure: T. Posas-Mendoza, None; C. McLeod, None; W. Davis, None; R. Quinet, None.

To cite this abstract in AMA style:

Posas-Mendoza T, McLeod C, Davis W, Quinet R. Etiologies and Management of Hemophagocytic Lymphohistiocytosis: Is It Time for an Updated Protocol and Targeted Treatments? [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/etiologies-and-management-of-hemophagocytic-lymphohistiocytosis-is-it-time-for-an-updated-protocol-and-targeted-treatments/. Accessed January 21, 2021.
  • Tweet
  • Email
  • Print
Save to PDF

« Back to ACR Convergence 2020

ACR Meeting Abstracts - https://acrabstracts.org/abstract/etiologies-and-management-of-hemophagocytic-lymphohistiocytosis-is-it-time-for-an-updated-protocol-and-targeted-treatments/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

ACR Convergence: Where Rheumatology Meets. All Virtual. November 5-9.

ACR Pediatric Rheumatology Symposium 2020

© COPYRIGHT 2021 AMERICAN COLLEGE OF RHEUMATOLOGY

Wiley

  • Home
  • Meetings Archive
  • Advanced Search
  • Meeting Resource Center
  • Online Journal
  • Privacy Policy
  • Permissions Policies
loading Cancel
Post was not sent - check your email addresses!
Email check failed, please try again
Sorry, your blog cannot share posts by email.
This site uses cookies: Find out more.