Indolent systemic mastocytosis (SM) patients have a varied clinical presentation, ranging from predominantly cutaneous symptoms to recurrent systemic symptoms (eg, flushing, palpitations, dyspepsia, diarrhea, bone pain) that can be severe and potentially life threatening (anaphylaxis). Mastocytosis patients without skin involvement pose a diagnostic challenge; a high index of suspicion is neede...

A case of systemic mastocytosis is described in which the finding on initial presentation was hepatosplenomegaly. No dermatological abnormality was present, and the bone marrow histology originally caused some confusion with primary myelofibrosis. The clinical course and the importance of distinguishing between these two diseases is discussed. The dermatological manifestation of systemic mastoc...

Background Mastocytosis is a disease characterized by abnormal growth and accumulation of mast cells (MC) in different tissues. The disease can be classified as cutaneous (CM), when MC infiltrate is limited to the skin, and systemic mastocytosis (SM) when MC proliferate in other organs such as the bone marrow, gastrointestinal tract, lung, liver, spleen or lymph-nodes. Clinical symptoms of CM a...

This presentation which addresses changes and updates in mastocytosis is based on the recently published International Consensus Classification of myeloid neoplasms (Arber DA, Orazi A, Hasserjian RP, et al. Blood 2022; 140: 1200–1228). Mastocytosis a neoplastic disease characterised by clonal proliferation mast cells, accumulate one or multiple organs. The includes three clinicopathologic types...

BACKGROUND Systemic sclerosis (SSc) is a complex autoimmune disease characterized by vascular alterations and autoimmune activation leading to widespread organ fibrosis. At the early stage of disease when organ involvement and extent of disease are emerging, mast cells may have some role, as implied by both symptoms and histologic evidence. CASE PRESENTATION A female patient diagnosed with cu...

The term "mastocytosis" denotes a heterogeneous group of disorders characterised by abnormal growth and accumulation of mast cells (MC) in one or more organ systems. Symptoms result from MC chemical mediator's release, pathologic infiltration of neoplastic MC in tissues or both. Multiple molecular, genetic and chromosomal defects seem to contribute to an autonomous growth, but somatic c-kit D81...

A 37-year-old man developed encephalopathy with prominent eosinophilia. Magnetic resonance imaging (MRI) revealed multiple T2-weighted high signal intensity lesions with dimeglumine gadopentetate (Gd-DTPA) enhancement on T1-weighted images, which were distributed in the cerebral cortex, thalamus, deep white matter and cerebellum. He was diagnosed as having systemic mastocytosis on the basis of ...

Systemic mastocytosis is an uncommon disorder of mast cell proliferation in connective tissues. Mast cell degranulation may occur on exposure to various stimuli and drugs. The release of histamine, heparin and vasoactive substances such as prostaglandin D2 may cause severe hypotension and other anaphylactoid manifestations. Anaesthetic management should include perioperative stabilization of ma...

Systemic mastocytosis (SM) is a condition associated with clonal neoplastic proliferation of mast cells. In up to 40% of systemic mastocytosis cases, an associated clonal hematological disease of non-mast cell lineage, such as acute myeloid leukemia (AML), is diagnosed before, simultaneously with, or after the diagnosis of SM. Herein, we report a case of a 30-year-old man diagnosed with AML wit...

We report a unique case of systemic mastocytosis (SM) diagnosed in an ileal pouch biopsy obtained from a 44-year-old woman with ulcerative colitis. She presented with intermittent abdominal pain and watery diarrhea that did not respond to antibiotic therapy. The pouch biopsy showed expansion of the lamina propria by aggregates of CD117 and CD25-positive abnormal mast cells. A subsequent bone ma...