Mast cells and mast cell neoplasia: a review*

Aberrant expression of CD30 in neoplastic mast cells in high-grade mastocytosis

“…[1][2][3] Indolent and aggressive variants of systemic mastocytosis occur. 4,5 So-called B and C findings have been defined to sub-classify systemic mastocytosis.…”

“…Indolent systemic mastocytosis patients with a silent clinical course have a normal or near-normal life expectancy. [1][2][3]5,[8][9][10] In contrast, patients with aggressive systemic mastocytosis or mast cell leukemia have a less favorable prognosis and show a poor response to conventional cytostatic drugs. 5,8,11 These patients are therefore candidates for experimental therapy [11][12][13][14] and several attempts have been made to identify novel therapeutic targets in neoplastic mast cells.…”

Increased Angiogenesis in the Bone Marrow of Patients with Systemic Mastocytosis

“…[1][2][3][4] Cutaneous mastocytosis (CM) is commonly diagnosed in childhood and has a favorable prognosis. 5 In a high percentage of cases with pediatric CM, spontaneous regression is found.…”

“…Systemic mastocytosis (SM) can develop at any age and is characterized by multiorgan involvement and disease persistence. [1][2][3][4] In most SM patients, somatic mutations in the kinase domain of c-kit are detectable. 6 -8 The majority of patients with SM show an indolent clinical course throughout decades.…”

“…6 -8 The majority of patients with SM show an indolent clinical course throughout decades. [1][2][3][4] However, in a smaller subset, progression to aggressive or even leukemic disease is seen. 1-4,9 -12 These patients acquire organopathy caused by progressive MC infiltrates in diverse organs such as the bone marrow (BM), liver, or skeletal system.…”