Journal issues > Issue 6, October 2024 > Systemic mastocytosis: Overview of recent updates and novel therapies

Systemic mastocytosis: Overview of recent updates and novel therapies

By: P. Sriskandarajah MD, MRCP, FRCPath, PhD, D.H. Radia MD, MRCP, FRCPath

SUMMARY

Systemic Mastocytosis (SM) is a rare, clinically heterogeneous haematological disorder. Over 90% of patients carry a mutation in the receptor tyrosine kinase KIT (i.e. KIT D816V) resulting in the clonal expansion of neoplastic mast cells (MCs) impacting multiple organs and leading to variable clinical presentations ranging from skin-limited disease to more aggressive variants associated with multi-organ dysfunction and reduced survival. Furthermore, the quality of life can be impaired by excess mediator release from neoplastic mast cells resulting in a significant symptom burden. Based on the above, there have been increasing efforts to develop novel, targeted therapies in order to improve quality of life while also affecting survival outcomes. This review will give an overview of the diagnostic work-up for SM patients as well as therapeutic management, including updates from recent clinical trials.

(BELG J HEMATOL 2024;15(6):216–24)

To read the full artice you have the following options:

* (only possible for healthcare professionals with RIZIV/INAMI/MATRICULE working in the field of Hematology in Belgium and Luxembourg with prescription authorisation)

Purchase this article for €3.03

** (for healthcare professionals with prescribing authorisation working outside the field of Hematology in Belgium and Luxembourg as well as those without prescribing authorisation working as healthcare professional in Hematology in Belgium and Luxembourg, plus persons without prescription authorisation working outside Belgium and Luxembourg, plus healthcare professionals with prescribing authorisation outside Belgium and Luxembourg plus non-healthcare professionals from outside or inside Belgium or Luxembourg)

Top