NextGen 2024: Refractory SJIA & MAS Session Part 3

NextGen 2024: Refractory SJIA & MAS Session Part 3

@SJIA_Foundation

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2 weeks ago 63

Science #sjiafoundation #sjia #MacrophageActivation #curesjia #StillDisease #Emapalumab

NextGen 2024: Refractory SJIA & MAS Session Part 3

@SJIA_Foundation2 weeks ago

TREATMENT PATTERNS AND OUTCOMES IN PATIENTS WITH
MACROPHAGE ACTIVATION SYNDROME SECONDARY TO STIL…

1/21

• Hemophagocytic lymphohistiocytosis (HLH) is a rare, frequently fatal, hyperinflammatory syndrome …

2/21

REAL-HLH: Study Objectives and Design
3
• The REAL-HLH study assessed clinical and demographic ch…

3/21

Demographics and Clinical Characteristics of Patients
with Still’s Disease (n = 10)
4
• Ten of …

4/21

Laboratory Parameter Values at Emapalumab Initiation
5
Laboratory marker/cytokine
Patients with …

5/21

HLH-related Therapies Used Prior to or Concurrently
with Emapalumab
6
• Emapalumab was initiate…

6/21

Emapalumab Treatment Duration and Dosing
7
‡Date of diagnosis was missing for one of the patients…

7/21

Change in Glucocorticoid Dose Over Time
8
Glucocorticoid dose reduced by approximately 32%
from…

8/21

Time to First Normalization of Laboratory Parameters
from Emapalumab Initiation
9
Median time t…

9/21

Time to Achieving Defined Laboratory Criteria
from Emapalumab Initiation
10
Parameter
Proporti…

10/21

Overall Survival from Emapalumab Initiation
11
• Median (range) duration of follow-up from
emap…

11/21

Emapalumab Treatment as a Bridge to HSCT
12
(1/4)
(4/10) • Four of 10 (40%) patients were consid…

12/21

• As with other retrospective chart review studies, data may not be uniformly available across all …

13/21

• This is the first study to report real-world treatment patterns and outcomes among patients with …

14/21

Phase II Open label trial of Emapalumab in MAS
Demographic (n=14)
Age, years, median (range) 11.0…

15/21

Emapalumab seems efficacious in MAS
Ferritin, platelets, liver enzymes, and LDH all
improve in 1…

16/21

IFNg biology shows rapid improvement on emapalumab

17/21

Conclusions
• Emapalumab has significant real world use experience as well as clinical trial evide…

18/21

REAL-HLH Study Group (Investigators)
19
Dr. Allyson Hays [CHILDREN’S MERCY HOSPITALS AND CLINICS]…

19/21

20/21

Laboratory Parameter Values at Time Closest to
Emapalumab Initiation: Still’s disease (n = 10)
2…

21/21

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NextGen 2024: Refractory SJIA & MAS Session Part 3

@SJIA_Foundation

Presentation from Dr. Ed Behrens: Treatment Paterns and Outcomes in Patients with Macrophage Activation Syndrome Secondary to Still's Disease Treated with Emapalumab

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#sjiafoundation #sjia #MacrophageActivation #curesjia #StillDisease #Emapalumab

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