Background Mastocytosis is a group of heterogeneous diseases characterized by an abnormal expansion and accumulation of mast cells (MCs) in different tissues. While systemic mastocytosis (SM) is defined by the accumulation of MCs in bone marrow (BM) and different tissues, cutaneous mastocytosis (CM) is characterized as an accumulation of MC in the skin with no other organ involvement. How gende...

T HE diagnostic value of serum α-protryptase levels in patients with suspected systemic mastocytosis was evaluated. The retrospective study included 22 patients with suspected mastocytosis and 30 with suspected anaphylaxis. All patients had high total serum tryptase levels of 20 ng/mL or greater and normal or slightly elevated serum β-tryptase levels--no higher than 5 ng/mL. Biopsy results were...

Mastocytosis is a clonal proliferation of mast cells that accumulates in one or more organs [1]. This disorder is heterogenous, ranging from skin lesion that may spontaneously regress to multiple organ lesions that show a highly aggressive course, and a recent World Health Organization Classification divides it into 7 subtypes: cutaneous mastocytosis, indolent systemic mastocytosis, systemic ma...

Systemic mastocytosis is a myeloproliferative disorder characterized by extracutaneous involvement of at least one organ. Although rare, infiltration of inflammatory mast cells within the portal vein may lead to obstruction of the sinusoids resulting in non-cirrhotic portal hypertension. We present a patient with known history of systemic mastocytosis with bone marrow involvement presenting wit...

The pathogenesis of mastocytosis is not well defined and thus treatment remains challenging and remains on a palliative basis. We present two cases (a 48-year-old woman and a 57-year-old man) with indolent systemic mastocytosis in whom omalizumab (anti-IgE) reduced gastrointestinal and cutaneous symptoms significantly. This observation provides additional insight into the effects of omalizumab ...

Systemic mastocytosis is a disease of mast cell proliferation that may be associated with hematologic disorders. There are no features on examination that allow the diagnosis of systemic disease, and mast cell-derived mediators, which may be elevated in urine or blood, may also be elevated in individuals with severe allergic disorders. Thus, the diagnosis usually depends on results of bone marr...

Systemic mastocytosis are rare neoplasms characterized by accumulation of mast cells in at least one internal organ. The majority of systemic mastocytosis patients carry KIT D816V mutation, which activates constitutively the KIT receptor. Patient with advanced forms of systemic mastocytosis, such as aggressive systemic mastocytosis or mast cell leukemia, are poorly treated to date. Unfortunatel...

Mastocytosis is a term used to describe a heterogeneous group of disorders characterized by clonal proliferation of mast cells in different organs. The organ most often affected is the skin. The World Health Organization classifies cutaneous mastocytosis into mastocytoma, maculopapular cutaneous mastocytosis, and diffuse mastocytosis. The systemic variants in this classification are as follows:...