Advanced
Systemic
Mastocytosis

Advanced SM is associated with shortened overall survival (OS)1,2

Advanced SM is a clonal mast cell neoplasm, causing shortened survival as well as symptom burden and impact1,3–5

Advanced SM has an estimated prevalence of ~1 in 100,000 and it commonly affects adults4

Drawing of a fictional patient’s medical file in a folder

Patients may experience debilitating mast cell mediator symptoms, such as rash and life-threatening anaphylaxis3

Additionally, patients with Advanced SM can experience organ damage, including ascites, osteolytic lesions, pleural effusion, liver dysfunction, weight loss, cytopenias and hypersplenism4–6

Historical median OS for patients with Advanced SM1*

ASM (n=41)
3.5 years
SM-AHN (n=138)
2 years
MCL (n=4)
< 6 months

Figure adapted from Lim et al. 2009
*OS was examined in a retrospective study at the Mayo Clinic between 1976 and 2007.1

Mechanism of Disease

Picture of KIT D816V mutation positive mast cell in bone marrow, with a close-up illustration of KIT D816V

Advanced SM is driven by KIT D816V mutation in ~95% of cases; however, until recently, there were no treatment options that selectively targeted this underlying mutation4–8

The KIT mutation constitutively activates downstream pathways regulating cellular functions including proliferation and survival of abnormal mast cells9,10

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Click here to learn more about AYVAKYT

About AYVAKYT

This medicinal product is subject to additional monitoring. This will allow quick identification of new safety information. Healthcare professionals are asked to report any suspected adverse reactions. See section 4.8 of the product SmPC for how to report adverse reactions.

References

  1. Lim KH, et al. Blood. 2009;113(23):5727–5736.
  2. Sperr WR, et al. Lancet Haematol. 2019;6(12):e638–e649.
  3. Jennings SV, et al. Immunol Allergy Clin North Am. 2018;38(3):505–525.
  4. Gulen T, et al. J Intern Med. 2016;279(3):211–228.
  5. Pardanani A. Am J Hematol. 2021;96(4):508–525.
  6. Verstovsek S. Eur J Haematol. 2013;90(2):89–98.

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