Systemic mast cell disease (SMCD)

Bone marrow mast cell morphologic features and hematopoietic dyspoiesis in systemic mast cell disease.

Systemic mast cell disease (SMCD) cannot be distinguished from reactive mastocytosis (RM) by quantitation of mast cells in aspirate smears, and few studies have analyzed systematically the morphologic features of mast cells in SMCD vs RM. In addition, although SMCD is associated with myeloproliferative disorders/myelodysplastic syndromes (MPD/MDS), it is not known whether subtle signs of dyspla...

Familial Occurrence of Systemic Mast Cell Activation Disease

Systemic mast cell activation disease (MCAD) comprises disorders characterized by an enhanced release of mast cell mediators accompanied by accumulation of dysfunctional mast cells. Demonstration of familial clustering would be an important step towards defining the genetic contribution to the risk of systemic MCAD. The present study aimed to quantify familial aggregation for MCAD and to invest...

Involvement of the small intestine in systemic mast cell disease.

A patient is reported with mast cell infiltration of the small intestine in the absence of the skin involvement characteristic of mast cell disease. She also had subtotal villous atrophy responsive to a gluten-free diet. Criteria for diagnosing mast cell disease of the small intestine are proposed. The literature of small intestinal mast cell disease is reviewed and the relationship to coeliac ...

Mast Cell Disease

Indolent systemic mast cell disease in adults: immunophenotypic characterization of bone marrow mast cells and its diagnostic implications.

The aim of the present study was to explore the diagnostic value of the immunophenotypic analysis of bone marrow mast cells (BMMC) in indolent systemic mast cell disease (SMCD) patients. For that purpose, a total of 10 SMCD patients and 19 healthy controls were analyzed. Our results show that BMMC from SMCD are different from normal BMMC with regard to both their light scatter and immunophenoty...