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Le Touquet – Paris-Plage, France

Fraitag S.,Hopital Necker Enfants Malades APHP
Revue Francophone des Laboratoires | Year: 2016

Mastocytoses are rare disorders linked to the anormal accumulation of clonal mast cells in the organs. The skin is the prefered target organ of mastocytoses. Children are more often affected with isolated cutaneous mastocytosis. The diagnosis of Mastocytosis may be difficult both clinically and histologically. Pathological mast cells can take on different aspects which make them difficult to differentiate from other resident skin cells. This is why some small associated signs are all the more important to detect because they can direct the pathologist toward theright diagnosis especially if the clinical presentation is not typical and the importance of the highlighting of mast cells by special stains, and especially immunohistochemistry using anti-c-KIT. The different clinico/pathological forms of cutaneous mastocytoses will be described. © 2016 Elsevier Masson SAS. Source

Canioni D.,Hopital Necker Enfants Malades APHP | Lhermitte L.,Hopital Necker Enfants Malades APHP
Revue Francophone des Laboratoires | Year: 2016

Mastocytosis is a heterogeneous group of rare, sporadic or familial diseases, due to a clonal proliferation of abnormal mast cells (MC) that accumulate in one or more organs. It is characterized by the presence of multifocal compact aggregates of abnormal MC. Mastocytosis is frequently associated with somatic activating point mutations within KIT among which the most frequent is the D816V mutation. The diagnosis of mastocytosis is based on the identification of abnormal MC associated with molecular and genetic criteria using the 2008 OMS classification of tumors of hematopoietic and lymphoid tissues.We describe the main cytological and histological features of systemic mastocytosis (SM) observed in hematology and in particular, the « classical » and more atypical features of bone marrow involvement in SM, the SM associated with hematological non-mast cell lineage diseases (SM-AHNMD) myeloid or non-myeloid, the mast cell leukemia, the involvement of lymph node and of spleen in MS and the mast cell sarcomas.Finally, we conclude on the respective role of bone marrow aspirate and bone marrow biopsy in the diagnosis of mastocytosis. © 2016 Elsevier Masson SAS. Source

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