Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 60-year-old male was bitten by a venomous snake (Vipera ammodytes) and gradually developed signs of an allergic reaction including generalized itching, generalized rash, and chest discomfort. This was followed by severe retrosternal pain with electrocardiographic evidence of an inferior myocardial ischemia progressing to acute myocardial infarction. Cardiac enzymes and troponin, serum tryptase, and histamine were elevated. Coronary arteriography showed normal coronary arteries. This is a characteristic type I variant of Kounis syndrome, which is the concurrence of acute coronary syndromes with conditions associated with mast cell activation including allergic or hypersensitivity reactions as well as anaphylactic or anaphylactoid reactions. This is the first report to show that viper bites can induce allergic angina and/or allergic myocardial infarction.
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PMID:Hypersersensitivity and Kounis syndrome due to a viper bite. 1661 60

Assessment of patients with unexplained hypotensive episodes in outpatient practice is often challenging, with an extensive differential diagnosis. The prevalence of systemic mast cell disease (MCD) is unknown, and the diagnosis is often elusive because serum and urine markers may become positive only after one of the self-limited, recurrent hypotensive episodes. Nevertheless, MCD is increasingly recognized as a cause of unexplained hypotension, secondary osteoporosis, and anaphylactic reactions to Hymenoptera stings. We describe a 38-year-old man who had a 15-year history of undiagnosed, recurrent hypotensive episodes with stereotypic symptoms. Extensive evaluation during these years was unrevealing. On physical examination, he appeared to be a healthy man with a prominent macular rash. Results of skin biopsy showed tryptase-positive mast cells. He had markedly elevated serum tryptase levels, and results of bone marrow biopsy revealed 10% mast cells; all these findings were consistent with indolent systemic mastocytosis. Key features in his history and physical examination prompted the conclusive testing. The most telling features were hypotension, tachycardia, and the rash (urticaria pigmentosa).
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PMID:Indolent systemic mastocytosis as the cause of a long history of unexplained hypotensive episodes. 1692 85

An important feature of chemokines is their ability to bind to the glycosaminoglycan (GAG) side chains of proteoglycans, predominately heparin and heparan sulfate. To date, all chemokines tested bind to immobilized heparin in vitro, as well as cell surface heparan sulfate in vitro and in vivo. These interactions play an important role in modulating the action of chemokines by facilitating the formation of stable chemokine gradients within the vascular endothelium and directing leukocyte migration, by protecting chemokines from proteolysis, by inducing chemokine oligomerization, and by facilitating transcytosis. Despite the importance of eotaxin in eosinophil differentiation and recruitment being well established, little is known about the interaction between eotaxin and GAGs and the functional consequences of such an interaction. Here we report that eotaxin binds selectively to immobilized heparin with high affinity (K(d) = 1.23 x 10(-8) M), but not to heparan sulfate or a range of other GAGs. The interaction of eotaxin with heparin does not promote eotaxin oligomerization but protects eotaxin from proteolysis directly by plasmin and indirectly by cathepsin G and elastase. In vivo, co-administration of eotaxin and heparin is able to significantly enhance eotaxin-mediated eosinophil recruitment in a mouse air-pouch model. Furthermore, when heparin is co-administered with eotaxin at a concentration that does not normally result in eosinophil infiltration, eosinophil recruitment occurs. In contrast, heparin does not enhance eotaxin-mediated eosinophil chemotaxis in vitro, suggesting protease protection or haptotactic gradient formation as the mechanism by which heparin enhances eotaxin action in vivo. These results suggest a role for mast cell-derived heparin in the recruitment of eosinophils, reinforcing Th2 polarization of inflammatory responses.
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PMID:Eotaxin selectively binds heparin. An interaction that protects eotaxin from proteolysis and potentiates chemotactic activity in vivo. 1738 13

Mastocytosis is a neoplastic disease involving mast cells (MC) and their CD34+ progenitors. Symptoms in mastocytosis are caused by biological mediators released from MC and/or the infiltration of neoplastic MC in various organs, the skin and the bone marrow being predominantly involved. A WHO consensus classification for mastocytosis exists, which is widely accepted and includes three major categories: (1) Cutaneous mastocytosis (CM), a benign disease in which MC infiltration is confined to the skin, is preferentially seen in young children and exhibits a marked tendency to regress spontaneously. (2) Systemic mastocytosis (SM) which is commonly diagnosed in adults and includes four major subtypes: (i) indolent SM (ISM, the most common form involving mainly skin and bone marrow); (ii) a unique subcategory termed SM with an associated non-mast cell clonal hematological disease (SM-AHNMD); (iii) aggressive SM usually presenting without skin lesions, and (iv) MC leukemia, probably representing the rarest variant of human leukemias. (3) The extremely rare localized extracutaneous MC neoplasms, either presenting as malignancy (MC sarcoma) or as benign tumor termed extracutaneous mastocytoma. Diagnostic criteria for mastocytosis are available and are widely accepted. SM criteria include one major criterion (multifocal compact tissue infiltration by MC) and four minor criteria: (1) prominent spindling of MC; (2) atypical immunophenotype of MC with coexpression of CD2 and/or CD25 (antigens which have not been found to be expressed on normal/reactive MC); (3) activating (somatic) point mutations of the c-kit proto-oncogene usually involving exon 17, with the imatinib-resistant type D816V being most frequent, and (4) persistently elevated serum tryptase level (>20 ng/ml). To establish the diagnosis of SM, at least one major and one minor criterion, or at least three minor criteria, have to be fulfilled. The natural clinical course of mastocytosis is variable. Most patients, in particular those with CM and ISM, remain in an indolent stage over many years or even decades, while others, in particular those with aggressive SM, SM-AHNMD, or mast cell leukemia, show a progressive course, usually with a fatal outcome.
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PMID:Mastocytosis: state of the art. 1758 83

A patient with severe coronary disease, who underwent inguinal hernia repair, suffered a cardiovascular collapse after anaesthetic induction and died. He had Kounis syndrome, which is the concurrence of acute coronary syndrome (ACS) with conditions associated with mast cell activation. We measured the serum tryptase level in this patient and in a group of ten other patients admitted to hospital with the diagnosis of ACS with ST elevation in order to determine the origin of these events.
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PMID:Serum tryptase levels in acute coronary syndromes with ST elevation. 1798 53

The WHO has published an updated classification of mastocytosis and the criteria for the diagnosis of systemic mastocytosis (SM). These include one major criterion, compact mast cell (MC) infiltrates in extracutaneous tissues, and four minor criteria, i.e. cytomorphologic atypia with spindling of MC (>25 %), detection of the activating somatic c-kit mutation D816 V in MC, aberrant expression of CD2 and/or CD25 on MC, and an elevated serum tryptase level (>20 ng/ml). Systemic mastocytosis is diagnosed when the major plus one minor, or three minor criteria are fulfilled. In the present study, we have established methods for the detection of CD25 and the c-kit mutation D816V in paraffin-embedded bone marrow trephine biopsy specimen of 57 patients with various subtypes of mastocytoses and 239 controls. While MCs in almost all patients with SM (55/57) expressed CD25, only 2/239 of the control samples contained CD25-positive MCs. With newly designed molecular pathological methods, c-kit codon 816 mutations were detected by "peptide nucleic acid" (PNA)-mediated PCR-clamping and/or analysis of microdissected MC in 52/57 cases with SM. All cases with detectable c-kit mutations also contained CD25-positive MC. The c-kit mutation D816 V was also detected in microdissected cells of associated hematologic neoplasias in 6/15 cases. With the methods established for the investigation of paraffine-embedded tissues, the pathologist plays a central role in the diagnosis of SM.
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PMID:[Immunohistochemical and molecular characterization of systemic mastocytoses]. 1803 98

Patients with systemic mastocytosis (SM) may acquire an associated hematologic non-mast cell (MC)-lineage disease (AHNMD). In most cases, a myeloid neoplasm is diagnosed, whereas the occurrence of a lymphoproliferative disease is an extremely rare event. We report on a patient with indolent SM associated with small lymphocytic lymphoma (SLL). The patient presented with lymphadenopathy, maculopapular exanthema, and elevated serum tryptase. The bone marrow biopsy showed focal MC aggregates together with SLL. As assessed by immunostaining, neoplastic MC were found to exhibit CD117 and CD25 but did not display CD5 or CD20, whereas SLL cells were found to coexpress CD5 and CD20 but did not express MC antigens. The KIT mutation D816V was detected in sorted CD34(+) cells and unfractionated marrow cells but not in CD5(+) SLL cells, confirming the coexistence of 2 distinct neoplasms.
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PMID:Indolent systemic mastocytosis associated with atypical small lymphocytic lymphoma: a rare form of concomitant lymphoproliferative disease. 1844 46

Selective ewe's or goat's milk allergy without cow's milk allergy often presents as a severe picture. Diagnosis and follow-up are facilitated by the use of laboratory tests. Serum tryptase is elevated after mast cell degranulation. Combined determination of serum tryptase and immunoglobulin-E levels are particularly useful in the work-up of anaphylaxis, should it have been fatal. A case of ewe's milk allergy without cow's milk allergy and its laboratory work-up are reported.
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PMID:[Fatal ewe's milk-induced anaphylaxis: laboratory work-up]. 1858 75

Mastocytosis is a rare disease characterized by an elevated whole body mast cell number. Anaphylaxis is a severe, generalized hypersensitivity reaction with rapid onset. The problem of anaphylaxis and mastocytosis is due to strongly increased mediator release from the elevated mast cell number during allergic reactions. This explains the much higher prevalence of anaphylaxis in mastocytosis than in the general population and its severe and sometimes fatal course. Because of the increased risk of anaphylaxis in mastocytosis, all patients with severe or recurrent anaphylaxis should be analyzed for underlying mastocytosis by estimation of baseline serum tryptase. If this is elevated, patients also should be tested via skin examination for cutaneous mastocytosis and with a bone marrow biopsy. All patients with mastocytosis and anaphylaxis must be instructed about avoiding the responsible elicitors and should carry an emergency kit with adrenaline for self-application. In mastocytosis patients with anaphylaxis due to Hymenoptera stings, venom immunotherapy is recommended for life.
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PMID:The problem of anaphylaxis and mastocytosis. 1906 27

A case of adult-onset mastocytosis is presented to illustrate the classification, signs, symptoms, workup, treatment, and prognosis for this unusual condition. Although there is no cure for mastocytosis, symptoms of histamine release can be minimized with oral antihistamines. Ongoing surveillance of organ systems affected remains important. Our patient's mast cell disease predisposed him to bone loss, but there was no evidence of disease beyond the skin. He has done well with continued follow up monitoring his serum tryptase and oral antihistamine treatment.
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PMID:Case report of mastocytosis in an adult. 1907 84


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