Mastocytosis is a rare disease of clonal hematological disorders characterized by a pathological accumulation of Mast Cells (MCs) in different tissues, with variable symptomatology and prognosis. Signs and symptoms of Systemic Mastocytosis (SM) are due to pathological infiltration of MCs and to the release of chemical mediators, mainly histamine. Patients with SM may also present with neurological symptoms or complications. The pathophysiology of these neurological disorders remains uncertain to this day, but it can be associated with the infiltration of tissue mastocytes, release of mastocytes' mediators or both. Moreover, there is a lot to understand about the role of neurological symptoms in SM and knowing, for example, what is the real frequency of neurological disorders in SM and if is present a relation between other SM subtypes, because it has been noted that the alteration of the histamine expression may be an initiating factor for susceptibility, gravity and progression of the epigenetic disease. In this review we explain the possible pathophysiological mechanism about neurological symptomatology found in some patients affected by SM, describing the role of histamine and its receptors in the nervous system and, in light of the results, what the future prospects may be for a more specific course of treatment.
Systemic mastocytosis: The roles of histamine and its receptors in the central nervous system disorders
Paola DI MAURO
Primo
Writing – Original Draft Preparation
;
2021-01-01
Abstract
Mastocytosis is a rare disease of clonal hematological disorders characterized by a pathological accumulation of Mast Cells (MCs) in different tissues, with variable symptomatology and prognosis. Signs and symptoms of Systemic Mastocytosis (SM) are due to pathological infiltration of MCs and to the release of chemical mediators, mainly histamine. Patients with SM may also present with neurological symptoms or complications. The pathophysiology of these neurological disorders remains uncertain to this day, but it can be associated with the infiltration of tissue mastocytes, release of mastocytes' mediators or both. Moreover, there is a lot to understand about the role of neurological symptoms in SM and knowing, for example, what is the real frequency of neurological disorders in SM and if is present a relation between other SM subtypes, because it has been noted that the alteration of the histamine expression may be an initiating factor for susceptibility, gravity and progression of the epigenetic disease. In this review we explain the possible pathophysiological mechanism about neurological symptomatology found in some patients affected by SM, describing the role of histamine and its receptors in the nervous system and, in light of the results, what the future prospects may be for a more specific course of treatment.File | Dimensione | Formato | |
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