Abstract
Mast cell disease is an epigenetically and genetically determined disease entity with very diverse clinical manifestations in potentially every system and tissue due to inap pro priate release of variable subsets of mast cell mediators together with accumulation of either morphologically normal or altered mast cells. Easy bruising, excessive bleeding, and aberrancies of erythropoiesis can frequently be observed in patients with mast cell disease. A thorough history, including a family history, will guide the appropriate work-up, and laboratory evaluations may provide clues to diagnosis. In recent years, our understanding of the involvement of coagulation and anticoagulant pathways, the fibrinolytic system, and erythropoiesis in the pathophysiology of mast cell disease has increased considerably. This review summarizes current knowledge of the impact of the disturbed hemostatic and erythropoietic balance in patients with mast cell disease and describes options of treatment.
Highlights
Mast cells (MCs) are hematopoietic tissue immune cells that act both as effector and regulatory cells (e.g., [1]) in adaptive and innate immunity (e.g., [2])
Primary MC disease comprises a group of historically defined different disease entities: systemic mastocytosis (SM), MC activation syndrome (MCAS), cutaneous mastocytosis (CM), MC sarcoma and hereditary alphatryptasemia (HAT)
In the present review we summarize the impact of primary MC disease on blood coagulation and erythropoiesis
Summary
Mast cells (MCs) are hematopoietic tissue immune cells that act both as effector and regulatory cells (e.g., [1]) in adaptive and innate immunity (e.g., [2]). SM) [1] or morphologically altered and immunohistochemically identifiable mutated mast cells (SM and MC leukemia), can affect single or multiple systems (though multisystem presentations are far more common), usually manifesting with symptoms in a subacute or chronic waxing/waning or recurrent manner ([10], further references therein). Due to both the widespread distribution of MCs in the organism and the great heterogeneity of aberrant mediator expression patterns, symptoms can involve virtually all organs and tissues; the clinical presentation of primary mast cell disease is very diverse (Table 1 in [1]). In the present review we summarize the impact of primary MC disease on blood coagulation and erythropoiesis
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