Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis

Escribano, Luís; Akin, Cem; Castells, Mariana; Schwartz, Lawrence B.

doi:10.2174/187152806775269303

Cited by 78 publications

(85 citation statements)

References 173 publications

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“…If these features are present (especially two or more of them) and symptoms are responsive to histamine receptor (HR) antagonists (HR1 or HR2 blockers) or MC stabilizing agents (e.g. cromolyn sodium or ketotifen), a primary MC disease (SM) or MCAS has to be considered [20,21,22]. A reliable marker in suspected (occult) SM is the basal serum tryptase level.…”

Section: Clinical Featuresmentioning

confidence: 99%

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Clinical and Laboratory Parameters of Mast Cell Activation as Basis for the Formulation of Diagnostic Criteria

Valent

Horny

Triggiani

et al. 2011

Int Arch Allergy Immunol

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Mast cell (MC) activation occurs in a number of different pathologic conditions. Acute activation is commonly seen in patients with allergic reactions, with consecutive massive release of vasoactive and proinflammatory mediator substances from MCs, leading to the clinical signs and symptoms of anaphylaxis. In these patients, serum tryptase concentrations usually increase subtantially above baseline levels. Chronic MC activation is more difficult to diagnose, especially when symptoms are mild or atypical, and no underlying disease is found. In these patients, serum tryptase levels usually are normal. In a smaller group of patients, tryptase levels are constantly elevated and may point to an occult form of mastocytosis. These patients have to be examined for MC monoclonality, other criteria of a primary MC disease, non-MC hematopoietic neoplasms, and reactive disorders producing chronic MC activation or MC accumulation. In most patients in whom MC activation is found, histamine-induced symptoms can be documented and usually respond to treatment with histamine receptor antagonists or MC stabilizers. If this is not the case, alternative explanations for symptoms and differential diagnoses have to be considered.

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Section: Clinical Featuresmentioning

confidence: 99%

“…Other patients suffer from symptoms of local MC activation, such as flushing, acute urticaria, angioedema, asthma, rhinitis, or gastrointestinal cramping [15,16,17,18,19,20,21,22]. In all these situations, the patient should be explored for signs of systemic MC activation and (occult) mastocytosis [23,24,25].…”

Section: Clinical Featuresmentioning

confidence: 99%

Clinical and Laboratory Parameters of Mast Cell Activation as Basis for the Formulation of Diagnostic Criteria

Valent

Horny

Triggiani

et al. 2011

Int Arch Allergy Immunol

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“…Thrombocytopenia and leukocytosis is also common. Eosinophilia can also be observed in approximately 15% of patients [23].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Anaphylaxis as a Manifestation of Systemic Mastocytosis: A Case Report and Review of Literature

Haddad¹,

Joukhadar²,

Chehata³

et al. 2017

J Clin Case Rep

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Introduction: Anaphylaxis is defined as "a serious, life-threatening generalized or systemic hypersensitivity reaction" and "a serious allergic reaction that is rapid in onset and might cause death". The determination of different mast cells (MC) mediators, such as serum and/or plasma histamine and tryptase, is proposed for the diagnosis of anaphylaxis. There are differences in the clinical presentation of anaphylaxis in patients with indolent systemic mastocytosis versus idiopathic anaphylaxis.

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“…Perhaps the easiest way to disrupt signaling mediated by NTAL/LAB/LAT2 may be by inhibiting the kinases responsible for NTAL/LAB/LAT2 phosphorylation, namely Lyn, Syk, and Kit. Inhibitors for all three kinases have been identified and, at least Gleevec, which can inhibit Kit, has been successfully utilized in the clinic for leukemic disorders (Escribano et al, 2006). Again the question of selectivity must be raised regarding the potential effects of these agents on other cells.…”

Section: Possible Medical Applicationmentioning

confidence: 99%

Ntal/Lab/Lat2

Iwaki

Jensen

Gilfillan

2007

The International Journal of Biochemistry & Cell Biology

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NTAL (non-T cell activation linker)/LAB (linker for activation of B cells), now officially termed LAT2 (linker for activation of T cells 2) is a 25-30 kD transmembrane adaptor protein (TRAP) associated with glycolipid-enriched membrane fractions (GEMs; lipid raft) in specific cell types of hematopoietic lineage. Tyrosine phosphorylation of NTAL/LAB/LAT2 is induced by FcεRI aggregation and Kit dimerization in mast cells, FcγRI aggregation in monocytes, and BCR aggregation in B cells. NTAL/LAB/LAT2 is also expressed in resting NK cells but, unlike the related TRAP, LAT, not in resting T cells. As demonstrated in monocytes and B cells, phosphorylated NTAL/LAB/LAT2 recruits signaling molecules such as Grb2, Gab1 and c-Cbl into receptorsignaling complexes. Although gene knock out and knock down studies have indicated that NTAL/ LAB/LAT2 may function as both a positive and negative regulator of mast cell activation, its precise role in the activation of these and other hematopoietic cells remains enigmatic.

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Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis

Cited by 78 publications

References 173 publications

Clinical and Laboratory Parameters of Mast Cell Activation as Basis for the Formulation of Diagnostic Criteria

Clinical and Laboratory Parameters of Mast Cell Activation as Basis for the Formulation of Diagnostic Criteria

Anaphylaxis as a Manifestation of Systemic Mastocytosis: A Case Report and Review of Literature

Ntal/Lab/Lat2

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