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mastocytosis/oedeem
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Exophthalmos, Diplopia, and Bilateral Eyelid Edema: Symptoms of Ocular Mastocytosis.
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Mastocytosis is characterized by clonal mast cell proliferation with accumulation within various organs and uncontrolled activation with excessive mast cell mediator release. Ocular manifestations have rarely been published. We describe a 63-year-old man with bilateral exophthalmos that led to the
Spontaneous antenatal resolution of canine hydrops fetalis diagnosed by ultrasound.
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A previously unreported syndrome of transient mid-gestational hydrops fetalis identified by ultrasound was diagnosed in 16 litters of 16 different dogs between November 1999 and May 2002. During this study period, a total of 161 canine pregnancies were diagnosed by ultrasound. A 17th litter of eight
[Recurrent syncope as a presenting symptom of systemic mastocytosis].
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A 48-year-old man presented with recurrent syncope which was preceded by facial edema and difficulty in breathing. Physical examination, laboratory tests, abdominal CT and bone scan were all within normal limits. Bone marrow biopsy was consistent with mastocytosis. Systemic mastocytosis consists of
[Colonic involvement in systemic mastocytosis (author's transl)].
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The most common gastrointestinal lesions encountered in systemic mastocytosis are the gastric, duodenal and small bowell lesions. The authors present a case of diffuse colonic involvement diagnosed on a double contrast examination. The radiological signs correlated with the endoscopical and
Renal involvement and favorable outcome in a child with mastocytosis
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Mastocytosis is a rare myeloproliferative disease in which mast cells abnormally accumulate in the skin, bone marrow, intestine, liver, spleen, and lymph nodes. Characterized by uncontrolled proliferation of aberrant mast cells, the disease can present either cutaneously or systemically. Mast cells
Intrahepatic cholestasis due to systemic mastocytosis: a case report and review of literature.
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A 35-yr-old female presented with symptoms of obstructive jaundice. Liver biopsy, bone marrow aspiration, and biopsy revealed systemic mastocytosis and acute myeloid leukemia. The liver biopsy specimen showed infiltration of mast cells within portal tracts with periductal and portal edema,
Tetany, malabsorption, and mastocytosis.
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Mastocytosis is characterized by increased proliferation of mast cells. Two patients had systemic mastocytosis involving the skin and gastrointestinal tract, complicated by malabsorption and tetany. Absorption studies in these patients suggested that the entire small bowel was involved and that the
Systemic mastocytosis in 16 dogs.
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The clinical and pathologic features of systemic mastocytosis in 16 dogs are reported. There was no apparent breed or sex predilection, and the median age at presentation was 9.5 years. In 14 of 16 cases there was a primary cutaneous mast cell tumor (MCT). When cutaneous tumor location was compared
[Systemic mast cell disease associated with cutaneous xanthomas and markedly elevated serum IgE].
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Systemic mast cell disease (SMCD) is a rare disease often associated with symptoms of general malaise, pruritus, diarrhea, vomiting, fever, urticaria pigmentosa, hepatosplenomegaly and lymphadenopathy. We reported a case of SMCD associated with cutaneous xanthoma and serum hyper IgE. Skin biopsy
Pulmonary mastocytosis and enhanced lung inflammation in mice heterozygous null for the Foxf1 gene.
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The Forkhead Box f1 (Foxf1) transcriptional factor (previously known as HFH-8 or Freac-1) is expressed in endothelial and smooth muscle cells in the embryonic and adult lung. To assess effects of Foxf1 during lung injury, we used CCl(4) and butylated hydroxytoluene (BHT) injury models. Foxf1(+/-)
The care pathway for children with urticaria, angioedema, mastocytosis.
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Cutaneous involvement characterized by urticarial lesions with or without angioedema and itch is commonly observed in routine medical practice. The clinical approach may still remain complex in real life, because several diseases may display similar cutaneous manifestations. Urticaria is a common
Novel approaches to treating advanced systemic mastocytosis.
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Mastocytosis is a myeloproliferative neoplasm characterized by expansion of abnormal mast cells (MCs) in various tissues, including skin, bone marrow, gastrointestinal tract, liver, spleen, or lymph nodes. Subtypes include indolent systemic mastocytosis, smoldering systemic mastocytosis and advanced
Masitinib for the treatment of systemic and cutaneous mastocytosis with handicap: a phase 2a study.
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Treatment options for patients suffering from indolent forms of mastocytosis remain inadequate with the hyperactivation of mast cells responsible for many of the disease's systemic manifestations. Masitinib is a potent and highly selective oral tyrosine kinase inhibitor. A combined inhibition of
A case of POEMS syndrome associated with essential thrombocythaemia and dermal mastocytosis.
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We describe a case of POEMS syndrome presenting with the recognized features of polyneuropathy, organomegaly, endocrine abnormalities, monoclonal protein, skin changes and anasarca. The patient was found to have both a solitary sclerotic plasmacytoma of the pelvis and evidence of Castleman's disease
Idiopathic Anaphylaxis: A Form of Mast Cell Activation Syndrome.
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Idiopathic anaphylaxis is a condition caused by paroxysmal episodes of sudden-onset multiorgan involvement variably including laryngeal edema, urticaria, bronchoconstriction, dyspnea, hypoxia, abdominal pain, nausea, vomiting, diarrhea, and hypotension. Rarely, the episodes can lead to
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