Indolent Systemic Mastocytosis with Elevated Serum Tryptase, Absence of Skin Lesions, and Recurrent Severe Anaphylactoid Episodes

Florian, Stefan; Krauth, Maria‐Theresa; Simonitsch‐Klupp, Ingrid; Sperr, Wolfgang R.; Fritsche-Polanz, Robert; Sonneck, Karoline; Födinger, Manuela; Agis, Hermine; Böhm, Alexandra; Wimazal, Friedrich; Horny, Hans‐Peter; Valent, Peter

doi:10.1159/000083954

Cited by 72 publications

(61 citation statements)

References 68 publications

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“…44 Treatment of allergies in mastocytosis should follow general guidelines, with the exception of allergen immunotherapy, for which there are scant data regarding safety and efficacy (except Hymenoptera venom allergy; see below). The incidence of anaphylaxis in adult mastocytosis patients ranges from 20% to 49%, [44][45][46] which is significantly higher than the general population. Anaphylaxis is significantly more frequent in ISM, particularly in the absence of MIS, as compared with CM.…”

Section: 29mentioning

confidence: 96%

“…47 In one study, virtually all patients had a baseline serum tryptase level .11.4 ng/mL, with a majority exhibiting levels .20 ng/mL. 46 Patients with recurrent, unexplained anaphylaxis without MIS and normal or slightly increased tryptase levels may have clonal mast cells (without pathognomonic clusters) in the BM. This can occur in up to 40% of patients.…”

Section: 29mentioning

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: 29mentioning

confidence: 96%

Section: 29mentioning

confidence: 99%

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

Pardanani

2013

Blood

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“…Tryptase levels may remain elevated in cases of late-onset, biphasic or protracted cell activation (23,24) and in association with an underlying mastocytosis (25). Discrimination between mature b tryptase and total serum tryptase is not only likely to result in greater specificity in the diagnosis of anaphylaxis but can also be helpful when anaphylaxis occurs in the setting of mastocytosis (26).…”

Section: Tryptasementioning

confidence: 99%

Anaphylaxis during anaesthesia: diagnostic approach

Ebo¹,

Fisher²,

Hagendorens³

et al. 2007

Allergy

195

197

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Anaesthesiologists administer several drugs while providing general anaesthesia. Many of these drugs can elicit adverse drug reactions that fall apart into two major types. First, reactions that are usually dose-dependent and related to the pharmacological properties of the drug and/or its metabolites. Second, reactions that are unrelated to the drug's pharmacological characteristics and that are less dose-dependent. These reactions comprise drug intolerance, idiosyncratic reactions and drug-induced immune-mediated allergic and nonimmune-mediated so-called pseudo-allergic or anaphylactoid reactions. The terms anaphylactic and anaphylactoid, however, have been used inconsistently in the literature. Therefore, the nomenclature task force set up by the European Academy of Allergy and Immunology (EAACI) has proposed that anaphylactic-type reactions should be reclassified into allergic anaphylaxis and nonallergic anaphylaxis. Allergic anaphylaxis being further subdivided in IgE-mediated and non-IgE-mediated reactions (1).The exact prevalence of anaphylaxis during anaesthesia is difficult to ascertain. The estimated overall frequency has been reported to vary between 1 in 3500 and 1 in 13 000 procedures in French series (2, 3) and between 1 in 10 000 and 1 in 20 000 in an Australian study (4). The clinical manifestation of these reactions is not infrequently an almost immediate generalized response with bronchospasm and hypotension. The degree of severity varies and does not allow differentiation between an IgE-mediated or non-IgE mediated reaction resulting from nonspecific mediator release (5). The mortality from these reactions is in the range from 3 to 6%, and an additional 2% of patients experience significant residual brain damage (4).Diagnosis of anaphylaxis during anaesthesia is not always straightforward. It can be hampered as a broad spectrum of different drugs can elicit heterogeneous allergic and nonallergic reactions with distinct and sometimes unclear pathological mechanisms. Problems are certainly compounded as multiple drugs need to be administered during general anaesthesia. In addition, nonanaesthesia related drugs or procedures (e.g. disinfection) are sometimes administered/performed in the perioperative period and can also be the cause of an allergic reaction.In addition, none of the available diagnostic tests demonstrates absolute accuracy. False-positive test Correct management of anaphylaxis during anaesthesia requires a multidisciplinary approach with prompt recognition and treatment of the acute event by the attending anaesthesiologist, and subsequent determination of the responsible agent(s) with strict avoidance of subsequent administration of all incriminated and/or cross-reacting compounds.However, correct identification of the causative compound(s) and safe alternatives is not always straightforward and, too often, not done.This review is not intended to discuss acute management of anaesthesia-related anaphylaxis but summarizes the major causes of anaphylaxis during anaesthesia and the di...

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“…In addition, two other subvariants of indolent forms of the disease have been recognized: well-differentiated SM (WDSM) [11,12] and ISMs [13,14].…”

Section: Discussionmentioning

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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Indolent Systemic Mastocytosis with Elevated Serum Tryptase, Absence of Skin Lesions, and Recurrent Severe Anaphylactoid Episodes

Cited by 72 publications

References 68 publications

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

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

Anaphylaxis during anaesthesia: diagnostic approach

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

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