Lee, JK

Vadas, P

Allergy, Asthma & Clinical Immunology

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An elusive case of systemic mastocytosis

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POSTER PRESENTATION Open AccessAn elusive case of systemic mastocytosisJK Lee1*, P Vadas2From Canadian Society of Allergy and Clinical Immunology Annual Scientific Meeting 2009Halifax, Canada. 22-25 October 2009A 40 year old woman with recurrent “idiopathic” ana-phylaxis was referred. Past history revealed a right ovar-ian mucinous cyst adenoma that was surgicallyremoved. Medications included celebrex and alesse.Six months following the oopherectomy at age 15, shebegan to experience anaphylaxis every 2 months. Typi-cally these occurred in late afternoon, not on an emptystomach, and half related to physical activity. Duringthree reactions she self administered epinephrine thatled to improvement within minutes. She had one bipha-sic reaction. She did recall being stung by a vespid butwas asymptomatic.Her two – three hour reactions consisted of abdom-inal pain, nausea, vomiting, diarrhea, weakness, pruritiswith flushing, and documented hypotension. Occasion-ally, there was nasal congestion, stridor, palpatations,presyncope. On two occasions she had syncope. Therewere no triggers including cholinergic, foods, andcatamenial.Physical examination was unremarkable. She neitherhad Darier’s sign nor urticaria pigmentosa.Skin prick testing to common allergens was negative.Her serum tryptase was 8.3 ug/L. Tryptase immediatelyafter a reaction was 33 ug/L. Normetanephrines, meta-nephrines, vanillymandelic acid, 5-hydroxyindoleaceticacid, 24 hour urine catecholamines for noradrenalineand dopamine, serum chromogranin A and calcitoninwere all normal. Her CT abdomen and pelvis wasnormal.She continued to have episodes on ranitidine 150mgand ketotifen 4mg twice daily as well as cetirizine 20mgand singulair 10mg once daily.On bone marrow biopsy she met three WHO minorcriteria for systemic mastocytosis: greater than 25% mastcells having cytologically atypical hypogranular spindleshapes; flow cytometry showing positive aberrant co-expression of CD25 and CD2 on mast cells; and muta-tional analysis of the c-kit gene being positive for theD816V C-KIT mutation. Tryptase at bone marrowaspiration was 7.6ug/L.In patients with idiopathic anaphylaxis, consider abone marrow biopsy for systemic mastocytosis in spiteof a negative physical exam and normal tryptase.Author details1Clinical Immunology and Allergy, University of Toronto, Canada. 2Division ofClinical Immunology and Allergy, St Michael’s Hospital, University of Toronto,Canada.Published: 12 May 2010doi:10.1186/1710-1492-6-S1-P24Cite this article as: Lee and Vadas: An elusive case of systemicmastocytosis. Allergy, Asthma & Clinical Immunology 2010 6(Suppl 1):P24.Submit your next manuscript to BioMed Centraland take full advantage of: • Convenient online submission• Thorough peer review• No space constraints or color figure charges• Immediate publication on acceptance• Inclusion in PubMed, CAS, Scopus and Google Scholar• Research which is freely available for redistributionSubmit your manuscript at www.biomedcentral.com/submit1Clinical Immunology and Allergy, University of Toronto, CanadaLee and Vadas Allergy, Asthma & Clinical Immunology 2010, 6(Suppl 1):P24http://www.aacijournal.com/content/6/S1/P24 ALLERGY, ASTHMA & CLINICAL IMMUNOLOGY© 2010 Lee and Vadas; licensee BioMed Central Ltd.

JK Lee

P Vadas

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POSTER PRESENTATION Open Access
An elusive case of systemic mastocytosis
JK Lee1*, P Vadas2
From Canadian Society of Allergy and Clinical Immunology Annual Scientific Meeting 2009
Halifax, Canada. 22-25 October 2009
A 40 year old woman with recurrent “idiopathic” ana-
phylaxis was referred. Past history revealed a right ovar-
ian mucinous cyst adenoma that was surgically
removed. Medications included celebrex and alesse.
Six months following the oopherectomy at age 15, she
began to experience anaphylaxis every 2 months. Typi-
cally these occurred in late afternoon, not on an empty
stomach, and half related to physical activity. During
three reactions she self administered epinephrine that
led to improvement within minutes. She had one bipha-
sic reaction. She did recall being stung by a vespid but
was asymptomatic.
Her two – three hour reactions consisted of abdom-
inal pain, nausea, vomiting, diarrhea, weakness, pruritis
with flushing, and documented hypotension. Occasion-
ally, there was nasal congestion, stridor, palpatations,
presyncope. On two occasions she had syncope. There
were no triggers including cholinergic, foods, and
catamenial.
Physical examination was unremarkable. She neither
had Darier’s sign nor urticaria pigmentosa.
Skin prick testing to common allergens was negative.
Her serum tryptase was 8.3 ug/L. Tryptase immediately
after a reaction was 33 ug/L. Normetanephrines, meta-
nephrines, vanillymandelic acid, 5-hydroxyindoleacetic
acid, 24 hour urine catecholamines for noradrenaline
and dopamine, serum chromogranin A and calcitonin
were all normal. Her CT abdomen and pelvis was
normal.
She continued to have episodes on ranitidine 150mg
and ketotifen 4mg twice daily as well as cetirizine 20mg
and singulair 10mg once daily.
On bone marrow biopsy she met three WHO minor
criteria for systemic mastocytosis: greater than 25% mast
cells having cytologically atypical hypogranular spindle
shapes; flow cytometry showing positive aberrant co-
expression of CD25 and CD2 on mast cells; and muta-
tional analysis of the c-kit gene being positive for the
D816V C-KIT mutation. Tryptase at bone marrow
aspiration was 7.6ug/L.
In patients with idiopathic anaphylaxis, consider a
bone marrow biopsy for systemic mastocytosis in spite
of a negative physical exam and normal tryptase.
Author details
1Clinical Immunology and Allergy, University of Toronto, Canada. 2Division of
Clinical Immunology and Allergy, St Michael’s Hospital, University of Toronto,
Canada.
Published: 12 May 2010
doi:10.1186/1710-1492-6-S1-P24
Cite this article as: Lee and Vadas: An elusive case of systemic
mastocytosis. Allergy, Asthma & Clinical Immunology 2010 6(Suppl 1):P24.
Submit your next manuscript to BioMed Central
and take full advantage of: 
• Convenient online submission
• Thorough peer review
• No space constraints or color figure charges
• Immediate publication on acceptance
• Inclusion in PubMed, CAS, Scopus and Google Scholar
• Research which is freely available for redistribution
Submit your manuscript at 
www.biomedcentral.com/submit
1Clinical Immunology and Allergy, University of Toronto, Canada
Lee and Vadas Allergy, Asthma & Clinical Immunology 2010, 6(Suppl 1):P24
http://www.aacijournal.com/content/6/S1/P24 ALLERGY, ASTHMA & CLINICAL 
IMMUNOLOGY
© 2010 Lee and Vadas; licensee BioMed Central Ltd.


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An elusive case of systemic mastocytosis

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