PMID- 31259747 OWN - NLM STAT- MEDLINE DCOM- 20200730 LR - 20200730 IS - 1473-6322 (Electronic) IS - 1473-6322 (Linking) VI - 19 IP - 5 DP - 2019 Oct TI - Mastocytosis presenting as insect anaphylaxis: gender differences and natural history. PG - 468-474 LID - 10.1097/ACI.0000000000000567 [doi] AB - PURPOSE OF REVIEW: Currently, there is strong evidence about an association between hymenoptera venom anaphylaxis (HVA) and mastocytosis. This review is focused on the most relevant clinical and biological features of systemic mastocytosis associated with HVA. RECENT FINDINGS: HVA is a relatively common complication that modifies the natural course of patients with mastocytosis, particularly men with indolent systemic mastocytosis without skin lesions (ISMs-) in whom HVA can be the presenting symptom in up to around one-half of the cases. Patients with ISMs- associated with HVA are typically males with cardiovascular symptoms in the absence of itching, urticaria, and angioedema during anaphylaxis. Noteworthy, ISMs- is characterized by a low bone marrow mast cell load and a low risk for disease progression. Early and more recent studies support that specific venom immunotherapy (VIT) is a well-tolerated and effective treatment in patients with mastocytosis. SUMMARY: VIT should be given life-long to all patients with mastocytosis and proven immunoglobulin E (IgE)-mediated HVA. In patients with negative venom skin test and undetectable IgE antibodies, additional studies such as component-based allergy testing might contribute to confirm an IgE-mediated mechanism of anaphylaxis in some cases, thus providing the indication of VIT. FAU - Alvarez-Twose, Ivan AU - Alvarez-Twose I AD - Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast), Hospital Virgen del Valle, Complejo Hospitalario de Toledo. AD - Spanish Network on Mastocytosis (REMA), Toledo, Spain. FAU - Matito, Almudena AU - Matito A AD - Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast), Hospital Virgen del Valle, Complejo Hospitalario de Toledo. AD - Spanish Network on Mastocytosis (REMA), Toledo, Spain. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Curr Opin Allergy Clin Immunol JT - Current opinion in allergy and clinical immunology JID - 100936359 RN - 0 (Arthropod Venoms) RN - 37341-29-0 (Immunoglobulin E) SB - IM MH - *Anaphylaxis/blood/immunology/therapy MH - Animals MH - Arthropod Venoms/*toxicity MH - *Desensitization, Immunologic MH - Female MH - Humans MH - *Hymenoptera MH - Immunoglobulin E/blood/immunology MH - Male MH - *Mastocytosis/blood/immunology/therapy MH - *Sex Characteristics EDAT- 2019/07/02 06:00 MHDA- 2020/07/31 06:00 CRDT- 2019/07/02 06:00 PHST- 2019/07/02 06:00 [pubmed] PHST- 2020/07/31 06:00 [medline] PHST- 2019/07/02 06:00 [entrez] AID - 10.1097/ACI.0000000000000567 [doi] PST - ppublish SO - Curr Opin Allergy Clin Immunol. 2019 Oct;19(5):468-474. doi: 10.1097/ACI.0000000000000567.