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Drug Anaphylaxis As Presentation of Systemic Mastocytosis: About 7 Case Reports
S28 Abstracts
SATURDAY
105
Drug Anaphylaxis As Presentation of Systemic Mastocytosis: About 7 Case Reports F. Codreanu, M. Morisset, J. M. Renaudin, A. Schuller, J. Mouzita, E. Beaudouin, D. A. Moneret-Vautrin, G. Kanny; University Hospital, Nancy, FRANCE. RATIONALE: Severe or recurrent drug anaphylactic shock can be the first presentation of a systemic mastocytosis. METHODS: We report 7 cases of drug anaphylaxis with increased baseline serum tryptase levels. Each patient had skin tests with the incriminated drug, skin and bone marrow biopsies and test for cKit mutation. RESULTS: Seven patients (mean age 51 615 years) presented anaphylactic adverse drug reactions (ADRs). Incriminated medications were: NSAID (2 cases), acetaminophen (2), aspirin (1), ampicillin (1), cefotetan (1) and ACE inhibitor (1). Baseline serum tryptase was elevated (75.5 6 46 mg/L; normal range < 13 mg/L). Skin tests were positive in 2/7 cases (ampicillin, cefotetan). Assessment of skin and bone marrow showed an increased number of mast cells respectively in 5/7 and 2/7 cases. A v816 mutation was observed in 5/7 cases. CONCLUSIONS: In anaphylactic ADRs, systematic baseline serum tryptase assay should be systematically performed in order to evidence systemic mastocytosis.
106
The Availability of EpiPensÒ Used by Students with Severe Food allergies in Michigan Schools D. Kassab1, E. A. Robinson2, A. Russell1, M. McMorris1; 1University of Michigan, Ann Arbor, MI, 2St. Joseph Mercy Hospital, Ann Arbor, MI. RATIONALE: To compare the use of EpiPensÒ (EP) for acute food allergies in Michigan schools before and after the enactment of House Bill 5087, passed in 2004, that allows children eight years of age and older with a history of food allergy reactions to carry their EP in school. METHODS: A 33 item on-line questionnaire, similar to one administered in 1999, and an emailed reminder, were sent to 3636 Michigan school principals concerning current practices regarding EP. Five hundred forty-one were completed. RESULTS: Sixty-three percent (95%CI: 59%-67%) of respondents were aware of House Bill 5087. Schools reported some EP’s in the front office 52%(95%CI: 47%-56%). Children self-carried EP 32% (95%CI: 28%36%) compared to 17% (95%CI: 13%-22%) in 1999. Lunchroom staffs were trained to administer EP in 28% (95%CI: 24%-32%) of schools compared to 6% (95%CI: 3%-10%) in 1999. School secretaries account for the largest proportion of staff members trained to administer EP (77%), followed by school administrators (70%), teachers (57%), school nurses (34%) and lunchroom staffs. CONCLUSIONS: A greater proportion of schools reported that students self carry and that lunchroom staffs are trained to administer EP than in the previous survey. A substantial number of respondents in this sample are not aware of House Bill 5087. A majority of schools sampled store at least some EP in the front office where they may not be accessible in an emergency. While these results may not be generalizable to all Michigan schools, the results are of concern, given the potential seriousness of acute food allergies. Funding: St. Joseph Mercy Hospital
107
Prevalence of Allergy and Anaphylaxis in 210 Patients with Mastocytosis. A Study From The Spanish Network on Mastocytosis (REMA) D. Gonza´lez de Olano1, M. B. de la Hoz Caballer1, R. Nun˜ez Lo´pez1, L. Sa´nchez Mun˜oz1, M. Cuevas Agustı´n1, M. Dieguez1, M. Castells2, L. Escribano Mora1; 1Hospital Ramo´n y Cajal, Madrid, SPAIN, 2Brigham and Women´s Hospital, Harvard Medical School, Boston, MA. RATIONALE: Mast cells play a key role in allergy and induce mediatorrelated symptoms. Mastocytosis is heterogeneous group of disorders characterized by an abnormal proliferation and accumulation of mast cells. Despite clinical similarities, few studies have addressed the comorbidity between both pathologies.
J ALLERGY CLIN IMMUNOL JANUARY 2007
A prospective study was carried out to calculate the prevalence of allergy in patients with mastocytosis and the impact of allergic diseases in mastocytosis. METHODS: According to established questionnaires, 210 patients (163 adults and 47 children) with cutaneous and systemic mastocytosis were asked for history of asthma, rhinitis, conjunctivitis, atopic dermatitis, urticaria and anaphylaxis. They also underwent a total IgE, PhadiatopÒ infant determination (which contains aeroallergens and food allergens), specific IgE to latex and to Anisakis simplex. Clinical symptoms directly related to mastocytosis were also recorded. RESULTS: The prevalence of atopy was 38.6%, as defined by presence of specific IgE. The prevalence of allergy as defined by clinical symptoms associated to specific IgE, was 23.9%. Total IgE levels of patients with allergy as compared to patients without allergy was significantly higher (median 58 kU/L vs 16.5 kU/L, p50.0001). Anaphylaxis was present in 36 patients, 9 of which were IgE-mediated. Males were predominantly affected by either allergy and anaphylaxis regarding females (61.5% vs 38.5% and 72% vs 28%, respectively). Mastocytosis associated symptoms were similar in patients with and without allergy, except for anaphylaxis. CONCLUSIONS: Except for the high frequency of anaphylaxis in the in the patients with allergy, the coexistence of allergy does not seem to influence mastocytosis-associated symptoms. Funding: This work was supported by grants from the Fondo de Investigaciones Sanitarias (FIS) of the Ministerio de Sanidad y Consumo of Spain (03/0770; 05/769 and REMA, G03/007) from the Comunidad Auto´noma de Madrid (GR/SAL/0133/2004), from the Fundacio´n MMA
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Anaphylaxis As An Initial Manifestation Of Cholangiocarcinoma P. Ngamjanyaporn1, S. Janwityanujit1, P. Dekumyoy2; 1Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, THAILAND, 2Department of Helminthology, Faculty of Tropical Medicine, Mahidol University, Bangkok, THAILAND. RATIONALE: Although Opisthorchis viverini[OV] is commonly associated with cholangiocarcinoma in Thailand, it has never been report as a cause of anaphylaxis. CASE REPORT: A 57-year old female was admitted to hospital after developing angioedema, urticaria, wheezing and hypotension. Immediate treatment included adrenaline, chlorpheniramine, ranitidine, dexamethasone and intubation. During admission, she developed urticarial rash after almost every meal. There was no rash if she was starving. Investigation showed normal CBC and mild transaminitis. Total IgE level was 1,306 kIU/L. Specific IgE to aeroallergens and food were all negative. After 1 week of investigation for cause of anaphylaxis, she developed progressive jaundice. Ultrasonography of upper abdomen showed inhomogeneous hyperechoic lesion at posteroinferior segment of right hepatic lobe and prominent common bile duct[CBD] with abrupt narrowing. Endoscopic retrograde cholangiopancreatography[ERCP] showed marked dilatation of both intrahepatic duct[IHD] and CBD with abrupt narrowing at distal part. Sphincterotomy was done and stent was applied, resulting in good drainage of bile and also diminution of urticarial symptom. Bile cytology demonstrated Opisthorchis ova. Diagnosis of cholangiocarcinoma was made based on ERCP finding. She developed two times of ascending cholangitis and expired four months after diagnosis. Her serum was sent for identification of specific IgE to Opisthorchis viverrini. By ELISA, IgE antibody to OV and snail antigens gave titers at 1:1,600 and 1:6,400. By immunoblot assay, patient’s and prototype serum demonstrated identical band of specific IgE to OV and snail antigens. CONCLUSIONS: This is a first case report of OV inducing both cholangiocarcinoma and IgE mediated anaphylaxis.
SATURDAY
105
Drug Anaphylaxis As Presentation of Systemic Mastocytosis: About 7 Case Reports F. Codreanu, M. Morisset, J. M. Renaudin, A. Schuller, J. Mouzita, E. Beaudouin, D. A. Moneret-Vautrin, G. Kanny; University Hospital, Nancy, FRANCE. RATIONALE: Severe or recurrent drug anaphylactic shock can be the first presentation of a systemic mastocytosis. METHODS: We report 7 cases of drug anaphylaxis with increased baseline serum tryptase levels. Each patient had skin tests with the incriminated drug, skin and bone marrow biopsies and test for cKit mutation. RESULTS: Seven patients (mean age 51 615 years) presented anaphylactic adverse drug reactions (ADRs). Incriminated medications were: NSAID (2 cases), acetaminophen (2), aspirin (1), ampicillin (1), cefotetan (1) and ACE inhibitor (1). Baseline serum tryptase was elevated (75.5 6 46 mg/L; normal range < 13 mg/L). Skin tests were positive in 2/7 cases (ampicillin, cefotetan). Assessment of skin and bone marrow showed an increased number of mast cells respectively in 5/7 and 2/7 cases. A v816 mutation was observed in 5/7 cases. CONCLUSIONS: In anaphylactic ADRs, systematic baseline serum tryptase assay should be systematically performed in order to evidence systemic mastocytosis.
106
The Availability of EpiPensÒ Used by Students with Severe Food allergies in Michigan Schools D. Kassab1, E. A. Robinson2, A. Russell1, M. McMorris1; 1University of Michigan, Ann Arbor, MI, 2St. Joseph Mercy Hospital, Ann Arbor, MI. RATIONALE: To compare the use of EpiPensÒ (EP) for acute food allergies in Michigan schools before and after the enactment of House Bill 5087, passed in 2004, that allows children eight years of age and older with a history of food allergy reactions to carry their EP in school. METHODS: A 33 item on-line questionnaire, similar to one administered in 1999, and an emailed reminder, were sent to 3636 Michigan school principals concerning current practices regarding EP. Five hundred forty-one were completed. RESULTS: Sixty-three percent (95%CI: 59%-67%) of respondents were aware of House Bill 5087. Schools reported some EP’s in the front office 52%(95%CI: 47%-56%). Children self-carried EP 32% (95%CI: 28%36%) compared to 17% (95%CI: 13%-22%) in 1999. Lunchroom staffs were trained to administer EP in 28% (95%CI: 24%-32%) of schools compared to 6% (95%CI: 3%-10%) in 1999. School secretaries account for the largest proportion of staff members trained to administer EP (77%), followed by school administrators (70%), teachers (57%), school nurses (34%) and lunchroom staffs. CONCLUSIONS: A greater proportion of schools reported that students self carry and that lunchroom staffs are trained to administer EP than in the previous survey. A substantial number of respondents in this sample are not aware of House Bill 5087. A majority of schools sampled store at least some EP in the front office where they may not be accessible in an emergency. While these results may not be generalizable to all Michigan schools, the results are of concern, given the potential seriousness of acute food allergies. Funding: St. Joseph Mercy Hospital
107
Prevalence of Allergy and Anaphylaxis in 210 Patients with Mastocytosis. A Study From The Spanish Network on Mastocytosis (REMA) D. Gonza´lez de Olano1, M. B. de la Hoz Caballer1, R. Nun˜ez Lo´pez1, L. Sa´nchez Mun˜oz1, M. Cuevas Agustı´n1, M. Dieguez1, M. Castells2, L. Escribano Mora1; 1Hospital Ramo´n y Cajal, Madrid, SPAIN, 2Brigham and Women´s Hospital, Harvard Medical School, Boston, MA. RATIONALE: Mast cells play a key role in allergy and induce mediatorrelated symptoms. Mastocytosis is heterogeneous group of disorders characterized by an abnormal proliferation and accumulation of mast cells. Despite clinical similarities, few studies have addressed the comorbidity between both pathologies.
J ALLERGY CLIN IMMUNOL JANUARY 2007
A prospective study was carried out to calculate the prevalence of allergy in patients with mastocytosis and the impact of allergic diseases in mastocytosis. METHODS: According to established questionnaires, 210 patients (163 adults and 47 children) with cutaneous and systemic mastocytosis were asked for history of asthma, rhinitis, conjunctivitis, atopic dermatitis, urticaria and anaphylaxis. They also underwent a total IgE, PhadiatopÒ infant determination (which contains aeroallergens and food allergens), specific IgE to latex and to Anisakis simplex. Clinical symptoms directly related to mastocytosis were also recorded. RESULTS: The prevalence of atopy was 38.6%, as defined by presence of specific IgE. The prevalence of allergy as defined by clinical symptoms associated to specific IgE, was 23.9%. Total IgE levels of patients with allergy as compared to patients without allergy was significantly higher (median 58 kU/L vs 16.5 kU/L, p50.0001). Anaphylaxis was present in 36 patients, 9 of which were IgE-mediated. Males were predominantly affected by either allergy and anaphylaxis regarding females (61.5% vs 38.5% and 72% vs 28%, respectively). Mastocytosis associated symptoms were similar in patients with and without allergy, except for anaphylaxis. CONCLUSIONS: Except for the high frequency of anaphylaxis in the in the patients with allergy, the coexistence of allergy does not seem to influence mastocytosis-associated symptoms. Funding: This work was supported by grants from the Fondo de Investigaciones Sanitarias (FIS) of the Ministerio de Sanidad y Consumo of Spain (03/0770; 05/769 and REMA, G03/007) from the Comunidad Auto´noma de Madrid (GR/SAL/0133/2004), from the Fundacio´n MMA
108
Anaphylaxis As An Initial Manifestation Of Cholangiocarcinoma P. Ngamjanyaporn1, S. Janwityanujit1, P. Dekumyoy2; 1Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, THAILAND, 2Department of Helminthology, Faculty of Tropical Medicine, Mahidol University, Bangkok, THAILAND. RATIONALE: Although Opisthorchis viverini[OV] is commonly associated with cholangiocarcinoma in Thailand, it has never been report as a cause of anaphylaxis. CASE REPORT: A 57-year old female was admitted to hospital after developing angioedema, urticaria, wheezing and hypotension. Immediate treatment included adrenaline, chlorpheniramine, ranitidine, dexamethasone and intubation. During admission, she developed urticarial rash after almost every meal. There was no rash if she was starving. Investigation showed normal CBC and mild transaminitis. Total IgE level was 1,306 kIU/L. Specific IgE to aeroallergens and food were all negative. After 1 week of investigation for cause of anaphylaxis, she developed progressive jaundice. Ultrasonography of upper abdomen showed inhomogeneous hyperechoic lesion at posteroinferior segment of right hepatic lobe and prominent common bile duct[CBD] with abrupt narrowing. Endoscopic retrograde cholangiopancreatography[ERCP] showed marked dilatation of both intrahepatic duct[IHD] and CBD with abrupt narrowing at distal part. Sphincterotomy was done and stent was applied, resulting in good drainage of bile and also diminution of urticarial symptom. Bile cytology demonstrated Opisthorchis ova. Diagnosis of cholangiocarcinoma was made based on ERCP finding. She developed two times of ascending cholangitis and expired four months after diagnosis. Her serum was sent for identification of specific IgE to Opisthorchis viverrini. By ELISA, IgE antibody to OV and snail antigens gave titers at 1:1,600 and 1:6,400. By immunoblot assay, patient’s and prototype serum demonstrated identical band of specific IgE to OV and snail antigens. CONCLUSIONS: This is a first case report of OV inducing both cholangiocarcinoma and IgE mediated anaphylaxis.