Mastocytosis and insect venom allergy

Bonadonna, Patrizia; Zanotti, Roberta; Müller, Ulrich

doi:10.1097/aci.0b013e32833b280c

Cited by 104 publications

(73 citation statements)

References 44 publications

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“…However, a significant but variable proportion of patients will have levels ,20 ng/mL, which creates a dilemma in terms of the cutoff level for BM biopsy. 59 A positive correlation between baseline serum tryptase and grade of the initial allergic reaction has been reported. 63 Sensitization to 4.…”

Section: -58mentioning

confidence: 97%

“…Management of Hymenoptera venom allergy and anaphylaxis (HVA): HVA is an IgE-mediated phenomenon; the presentation can range from large local reactions to anaphylaxis that may be fatal. 59 In a study of 74 adult mastocytosis patients, the most common trigger for severe anaphylaxis was Hymenoptera stings. 45 In another study, 12% of patients with HVA, the majority with anaphylaxis, had an elevated baseline serum tryptase level.…”

Section: -58mentioning

confidence: 99%

“…64,65 Given the likelihood that HVA is IgE mediated in most mastocytosis patients, venom immunotherapy has been proposed as a treatment of choice, albeit with important caveats. 59,[66][67][68] General guidelines for the diagnosis and management of HVA are available, although they are not specific to mastocytosis patients. 69 I pursue full investigation for SM including BM biopsy in patients with anaphylaxis after Hymenoptera stings, regardless of tryptase level or presence of MIS.…”

Section: Malabsorption With Weight Loss Due To Gastrointestinal Mast mentioning

confidence: 99%

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How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage)

Pardanani

2013

Blood

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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 needed in those with mast cell–degranulation symptoms, including anaphylaxis following Hymenoptera stings or other triggers. Modern-era molecular and flow-cytometric diagnostic methods are very sensitive and can detect minimal involvement of bone marrow with atypical/clonal mast cells; in some cases, full diagnostic criteria for SM are not fulfilled. An important aspect of treatment is avoidance of known symptom triggers; other treatment principles include a stepwise escalation of antimediator therapies and consideration of cytoreductive therapies for those with treatment-refractory symptoms. The perioperative management of mastocytosis patients is nontrivial; a multidisciplinary preoperative assessment, adequate premedications, and close intra- and postoperative monitoring are critical. Smoldering mastocytosis is a variant with high systemic mast cell burden. While its clinical course can be variable, there is greater potential need for cytoreductive therapies (eg, interferon-alpha, cladribine) in this setting. A systematic approach to the diagnosis and treatment of indolent SM using a case-based approach of representative clinical scenarios is presented here.

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Section: -58mentioning

confidence: 97%

Section: -58mentioning

confidence: 99%

Section: Malabsorption With Weight Loss Due To Gastrointestinal Mast mentioning

confidence: 99%

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How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage)

Pardanani

2013

Blood

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“…Some of the comorbidities represent classical combinations representing a high-risk situation for the occurrence of life-threatening MCA (anaphylaxis). One typical example is the presence of an allergy against hymenoptera venom(s) in patients with mastocytosis (23,(65)(66)(67)(68). In these patients, insect stings can lead to extremely severe reactions, and cases of death have been reported in the literature (65)(66)(67)(68).…”

Section: The Impact Of Comorbidities In Patients With Mcasmentioning

confidence: 99%

Mast cell activation syndromes: definition and classification

Valent

2013

Allergy

104

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Mast cell activation (MCA) occurs in a number of different clinical conditions, including IgE-dependent allergies, other inflammatory reactions, and mastocytosis. MCA-related symptoms may be mild, moderate, severe, or even life-threatening. The severity of MCA depends on a number of different factors, including genetic predisposition, the number and releasability of mast cells involved in the reaction, the type of allergen, presence of specific IgE, and presence of certain comorbidities. In severe reactions, MCA can be documented by a substantial increase in the serum tryptase level above baseline. When symptoms are recurrent, are accompanied by an increase in mast cell-derived mediators in biological fluids, and are responsive to treatment with mast cell-stabilizing or mediator-targeting drugs, the diagnosis of mast cell activation syndrome (MCAS) is appropriate. Based on the underlying condition, these patients can further be classified into i) primary MCAS where KIT-mutated, clonal mast cells are detected, ii) secondary MCAS where an underlying inflammatory disease, often in the form of an IgE-dependent allergy, but no KIT-mutated mast cells, is found, and iii) idiopathic MCAS, where neither an allergy or other underlying disease, nor KITmutated mast cells are detectable. It is important to note that in many patients with MCAS, several different factors act together to lead to severe or even lifethreatening anaphylaxis. Detailed knowledge about the pathogenesis and complexity of MCAS, and thus establishing the exact final diagnosis, may greatly help in the management and therapy of these patients.Mast cells are tissue-fixed effector cells of allergic and other inflammatory reactions (1-5). In common with blood basophils, mast cells express high-affinity IgE-binding sites and store numerous proinflammatory and vasoactive mediators in their metachromatic granules (1-3). During a severe anaphylactic reaction, allergen-induced cross-linking of IgE-binding sites on mast cells is followed by an explosive release of granular mediators (1-3, 5-7). In addition, activated mast cells release newly synthesized membrane-derived (lipid) mediators of allergic reactions into the extracellular space. Blood basophils also participate in allergic and other inflammatory reactions in the same way as mast cells (1,8,9). However, not all allergic reactions involve both cell types, even if the reaction is systemic. In addition, some of the mediators relevant to anaphylactic reactions are produced and released primarily in mast cells but not in basophils, or only in blood basophils but not in tissue mast cells.The capacity of mast cells and basophils to release mediators of anaphylaxis in response to cell activation, also termed 'releasability', depends on a number of different factors, including the primary underlying disease, number and type of (pre)activated receptors and signaling cascades, and the genetic background (9-13). The severity of an anaphylactic reaction is determined by additional factors, including the number...

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“…A generally accepted risk factor for recurrent severe anaphylaxis in SM is a known allergy against hymenoptera venom. In fact, various studies have shown that venom allergic patients with SM have a particularly high risk for developing severe, life-threatening anaphylactic events after hymenoptera stings [39][40][41][42][43][44].…”

Section: The Risk Of Severe Anaphylaxis (Mcas) In Mastocytosismentioning

confidence: 99%