Prevalence of mastocytosis and Hymenoptera venom allergy in the United States

Schuler, Charles F.; Volertas, Sofija; Khokhar, Dilawar; Yüce, Hüseyin; Chen, Lu; Başer, Onur; Montejo, Jenny M.; Akin, Cem

doi:10.1016/j.jaci.2021.04.013

Cited by 17 publications

(8 citation statements)

References 38 publications

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“…Although a recent study showed a lower prevalence of MCD among the U.S. population compared to Europe, that study was highly susceptible to reporting bias [81]. The incidence of side effects during VIT in mastocytosis patients has been noted in up to 18.9% [82].…”

Section: Adverse Side Effects and Risk Factorsmentioning

confidence: 94%

Updates and Recent Advances on Venom Immunotherapy

Floyd

Adams

Golden

2023

Curr Treat Options Allergy

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Purpose of Review Venom immunotherapy has been utilized to treat Hymenoptera venom allergy since the 1920s. Over the last century, significant advances in the fields of immunology and genetics have led to improvements in the practice of venom immunotherapy. This review encompasses recent advances in the use of venom immunotherapy to provide precise, patient-centered care. Recent Findings Research about the mechanism of action of venom immunotherapy continues to highlight the modification of both the innate and adaptive immune systems. Molecular techniques have allowed for the identification of specific venom allergens to improve the diagnostic accuracy and safety of venom immunotherapy. Research continues to support the safety of accelerated schedules which can impact the cost, adherence, and quality of life for patients receiving this treatment modality. Finally, significant advances have led to the elucidation of risk factors that place patients at risk for reactions during and after venom immunotherapy. Creation of risk profiles for venom-allergic patients can thus inform the process of immunotherapy in order to provide personalized and precise care. Summary Significant progress in the use of venom immunotherapy makes the practice a dynamic and active field for continued research. Future research needs to build on these recent advances to continue to optimize and enhance this life-saving treatment.

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Section: Adverse Side Effects and Risk Factorsmentioning

confidence: 94%

Updates and Recent Advances on Venom Immunotherapy

Floyd

Adams

Golden

2023

Curr Treat Options Allergy

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“…23 The reported prevalence of SM in the general population ranges from 10 to 17.2 per 100.000 inhabitants and is far higher among patients with HVA, with reported rates varying between reports and by geographic area (1.8% in the US, 3.8% in Israel, and 1-7.9% in European cohorts). [24][25][26][27][28] According to the 2016 WHO classification, a diagnosis of SM is made if one major and one minor or three minor criteria are met; however, discoveries over the past few years have resulted in a push towards an updated set of diagnostic criteria (see Table 1). 23,29 In addition to the established disease subsets mentioned above, two distinct entities of interest have recently been identified; namely, monoclonal mast cell activation syndrome (MMAS) and bone marrow mastocytosis (BMM).…”

Section: Key Messagesmentioning

confidence: 99%

Clonal mast cell disorders and hereditary α‐tryptasemia as risk factors for anaphylaxis

Kačar

Rijavec

Šelb

et al. 2023

Clin Experimental Allergy

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The association between Hymenoptera venom‐triggered anaphylaxis (HVA) and clonal mast cell‐related disorders (cMCD) has been known for decades. However, recent breakthroughs in peripheral blood screening for KIT p.D816V missense variant have revealed the true extent of this clinical association whilst adding to our understanding of the underlying aetiology. Thus, recent large studies highlighted the presence of KIT p.D816V among 18.2% and 23% of patients with severe Hymenoptera venom‐triggered anaphylaxis. A significant proportion of those patients have normal serum basal tryptase (BST) levels, with no cutaneous findings such as urticaria pigmentosa or other systemic findings such as organomegaly that would have suggested the presence of cMCD. These findings of an increased prevalence suggest that the impact of cMCD on anaphylaxis could be clinically underestimated and that the leading question for clinicians could be changed from ‘how many patients with cMCD have anaphylaxis?’ to ‘how many patients with anaphylaxis have cMCD?’. The discovery of hereditary α‐tryptasemia (HαT)—a genetic trait caused by an increased copy number of the Tryptase Alpha/Beta 1 (TPSAB1) gene‐, first described in 2016, is now known to underlie the majority of cases of elevated BST outside of cMCD and chronic kidney disease. HαT is the first common heritable genetic modifier of anaphylaxis described, and it is associated with increased risk for severe HVA (relative risk = 2.0), idiopathic anaphylaxis, and an increased prevalence of anaphylaxis in patients with cMCD, possibly due to the unique activity profile of α/β ‐tryptase heterotetramers that may potentiate immediate hypersensitivity reaction severity. Our narrative review aims to highlight recent research to have increased our understanding of cMCD and HαT, through recent lessons learned from studying their association with HVA. Additionally, we examined the studies of mast cell‐related disorders in food and drug allergy in an effort to determine whether one should also consider cMCD and/or HαT in cases of severe anaphylaxis triggered by food or drugs.

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“…Nine out of 161 (5.6%) patients undergoing venom immunotherapy (VIT) had basal tryptase >11.4 ng/mL, and 3 (1.8%) had a clonal mast cell disorder. 24 …”

Section: Hymenoptera Venom Allergy and Mast Cell Activation Syndromesmentioning

confidence: 99%

Treatment of Indolent and Advanced Systemic Mastocytosis

Buonomo

Nucera

Criscuolo³

2022

Mediterr J Hematol Infect Dis

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Management of Indolent and Smoldering SM is focused on preventing anaphylactic reactions, identifying and avoiding symptom triggers. Skin and gastrointestinal symptoms are managed with H1- and H2-antihistamines. When skin symptoms are not adequately controlled, leukotriene antagonists and oral psoralen combined with ultraviolet therapy may be added. Proton pump inhibitors, sodium cromolyn and oral corticosteroids may be added for gastrointestinal symptoms. Patients should be prescribed with self-injectable epinephrine and trained to treat recurrent cardiovascular symptoms or anaphylaxis. Depression and cognitive impairment require a psychiatric evaluation for tailored treatment. Bone involvement is managed with bisphosphonates, and eventually interferon. Omalizumab is effective on all vasomotor symptoms, including anaphylaxis, but not on respiratory, musculoskeletal, and neuropsychiatric symptoms. A cytoreductive treatment is not recommended unless anti-mediator therapy has failed. Venom immunotherapy is mandatory for patients with hymenottera venom allergy. There is not a curative option for patients with Advanced SM. The available therapeutic options include tirosin-kinase inhibitors and cladribine, with variable duration and extent of response. Imatinib mesylate was the first drug approved for SM lacking the cKIT D816V mutation; dasatinib and nilotinib are not effective. Midostaurin is active on both wild type and mutant cKIT D816V while Avapritinib is a selective cKIT D816V inhibitor: they are approved for treatment of Advanced SM. Cladribine is a purine analogue with significant activity against monocytes that were thought to have a common progenitor with mast cells. Allogeneic stem cell transplantation is usually performed in younger selected patients. MASTOCYTOSIS; THERAPY; SKIN INVOLVEMENT, SYSTEMIC MASTOCYTOSIS; TYROSINKINASEINHIBITORS; MIDOSTAURIN.

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Prevalence of mastocytosis and Hymenoptera venom allergy in the United States

Cited by 17 publications

References 38 publications

Updates and Recent Advances on Venom Immunotherapy

Updates and Recent Advances on Venom Immunotherapy

Clonal mast cell disorders and hereditary α‐tryptasemia as risk factors for anaphylaxis

Treatment of Indolent and Advanced Systemic Mastocytosis

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