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Time is the enemy when it comes to pHLH

Patients often have less than 2 months to live without timely diagnosis and treatment 1

Primary hemophagocytic lymphohistiocytosis (pHLH) requires immediate treatment to control hyperinflammation and prevent irreversible organ damage. 2,3

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If the clinical signs are pointing to pHLH in a patient, consult with a hematologist/oncologist

When diagnosed early, pHLH can be treatable 4

From diagnosis to hematopoietic stem cell transplant (HSCT)

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    Diagnosis confirmation: Once a clinician confirms the pHLH diagnosis, immediate treatment is initiated, regardless of genetic confirmation (about 1-3 days). 5,6

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    Patient stabilization: Pharmacologic therapy is started to subdue hypercytokinemia, reduce hyperinflammation, and prevent irreversible organ damage. 3,5

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    HSCT: After pharmacologic therapy, treatment typically involves HSCT, the primary goal for most patients with pHLH. 3,5

    The 5-year overall survival rate of patients who are able to undergo HSCT is 66% 4

Know the initial signs.
Refer to a hematologist/oncologist

References: 1. George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med . 2014;5:69-86. doi:10.2147/JBM.S46255 2. 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 3. Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr . 2007;166(2):95-109. doi:10.1007/s00431-006-0258-1 4. Bergsten E, Horne A, Myrberg IH, et al. Stem cell transplantation for children with hemophagocytic lymphohistiocytosis: results from the HLH-2004 study. Blood . 2020;4(15):3754-3766. doi:10.1182bloodadvances.2020002101 5. 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 6. Data on file. Stockholm, Sweden: Swedish Orphan Biovitrum AB.