Managing HLH
Two main management goals for patients with HLH
Hemophagocytic lymphohistiocytosis (HLH) requires appropriate recognition and timely treatment to prevent irreversible organ damage.1
Controlling hyperinflammation
Limiting toxicities and further organ damage
Addressing the trigger
For patients whose testing results suggest a genetic etiology for their HLH, the ultimate goal is hematopoietic stem cell transplantation, which is the only curative treatment.1,2
For patients whose testing results suggest an alternate trigger such as infection, malignancy, or rheumatologic disease, the underlying condition should be treated first to see if the hyperinflammation resolves.2
Treatment approaches for controlling hyperinflammation in primary HLH
First-line therapy for patients with HLH involves broad immunosuppression using etoposide and steroids.3
In cases of incomplete response to first-line therapy, it is important to treat aggressively with other available options.3
For additional guidance about managing HLH in your patients, consider consulting an expert.
The INTO-HLH Registry
INTO-HLH, which stands for Insight into the Natural History and Treatment Outcomes of Hemophagocytic Lymphohistiocytosis, is a patient registry for people diagnosed with HLH in North America. This is a collaborative effort with Cincinnati Children’s Hospital Medical Center, Texas Children’s Hospital, and Sobi North America as the industry sponsor. The aim of this registry is to learn more about HLH, including the impact of this condition, treatment options, and associated outcomes.
The INTO-HLH Registry will collect information on HLH from patients’ medical records based on their routine clinical care before the onset of the condition and at least 5 years after the end of treatment. Data collected will improve the understanding of HLH with the aim of achieving better patient outcomes.
References: 1. Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041-4052. doi:10.1182/blood-2011-03-278127 2. George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014;5:69-86. doi:10.2147/JBM.S46255 3. Jordan MB, Allen CE, Greenberg J, et al. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer. 2019;66(11):e27929. doi:10.1002/pbc.27929
