Systemic Mastocytosis: Retrospective Review of a Decade's Clinical Experience at the Brigham and Women's Hospital

Abstract

The clinical experience with a group of 21 patients with systemic mastocytosis followed at our institution is summarized. Cutaneous and gastrointestinal symptoms and findings were the most prominent chronic manifestations; episodic vascular collapse was the most dramatic acute event. All patients had indolent mastocytosis. There was no mortality.

Increased Angiogenesis in the Bone Marrow of Patients with Systemic Mastocytosis

“…20 -22 SM is a clonal hematological disease that almost invariably affects the BM. [1][2][3][4][11][12][13][14]24 In the present study, we show that the BM MVD is significantly higher in SM compared to CM/UP or controls. This observation is novel and is in line with the notion that SM is a neoplastic hematological disease.…”

“…[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.…”

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

“…2,[5][6][7][8][9][10][11]13,14,[16][17][18][21][22][23]26,30,31 Malabsorption, which occurred in two patients in our series is well described in systemic mastocytosis, 2,5,7,9,10,16,23,30,31 occurring in 5-30% of patients. 30,32 While all patients in our series were women, the literature indicates an equal sex distribution for systemic mastocytosis in general 33 and for those cases with direct gastrointestinal involvement. [5][6][7][8][9][10][11]13,[16][17][18][19][21][22][23]31 Endoscopically and grossly, the most frequent and distinctive finding in our series was that of mucosal nodularity (present in four of five patients).…”