Title:
Systemic Mastocytosis in Children: About a Case

Authors:
Fadoua ELFARSSANI, Raihane Bahri, Adil JAHDAOUI, Hicham Yahyaoui, Mustapha AITAMEUR, Mohammed CHAKOUR, Morocco

Abstract:

Mastocytosis is a group of rare pathologies linked to the clonal accumulation of abnormal mast cell infiltrates in one or more organs. Their pathophysiology is dominated by activating mutations affecting C-Kit (Stem Cell Factor receptor).
There are many clinical forms, ranging from isolated cutaneous mastocytosis mainly affecting children, to aggressive systemic mastocytosis described mainly in adults with predilective bone marrow involvement.
According to the 2016 WHO classification of hematologic malignancies, systemic mastocytosis is considered a new entity of "myeloid neoplasias and acute leukemias" which combines cytology (abnormal mast cells) with other genetic and molecular criteria.

DOI: http://dx.doi.org/10.51505/IJMSHR.2021.5103