Clonal mast cell disorders in patients with systemic reactions to Hymenoptera stings and increased serum tryptase levels.
BACKGROUND
Anaphylaxis after Hymenoptera stings has been reported in subjects with mastocytosis, but few data exist regarding disease prevalence in populations allergic to these insects.


OBJECTIVE
The incidence of clonal mast cell (MC) disorders in subjects with both systemic reactions to Hymenoptera stings and increased serum baseline tryptase (sBT) levels was assessed by using bone marrow (BM) aspirates and biopsy specimens.


METHODS
Subjects with a history of a systemic reaction caused by a Hymenoptera sting underwent the standard diagnostic work-up for Hymenoptera allergy, and sBT levels were measured. Subjects with an increased sBT level had BM evaluation that included histology/cytology, flow cytometry, and detection of KIT mutations.


RESULTS
Forty-four (11.6%) of 379 subjects with systemic reactions had increased sBT levels (>11.4 ng/mL), and 31 (70.5%) of these had a history of anaphylaxis. Thirty-four subjects with increased sBT levels underwent a BM analysis. Histology detected diagnostic or subdiagnostic MC infiltrates in 22 (65%) of 34 patients. Abnormal MCs were identified by means of flow cytometry and cytology in 26 (78.8%) of 33 and 20 (58.8%) of 34 subjects, respectively. A KIT mutation was detected in 17 (54.8%) of 31 subjects. The diagnosis was indolent systemic mastocytosis in 21 (61.7%) of 34 subjects and monoclonal MC activation syndrome in 9 (26.5%) of 34 subjects. All subjects with anaphylaxis had one of those 2 disorders.


CONCLUSIONS
The concomitant presence of systemic reactions (especially anaphylaxis) after Hymenoptera stings and increased sBT levels strongly suggests that a BM examination is indicated for the diagnosis of clonal MC disease.
Bonadonna
Patrizia
P
Allergy Unit, Verona General Hospital, Verona, Italy.
Perbellini
Omar
O
Passalacqua
Giovanni
G
Caruso
Beatrice
B
Colarossi
Sabrina
S
Dal Fior
Daniela
D
Castellani
Luca
L
Bonetto
Chiara
C
Frattini
Francesco
F
Dama
Annarita
A
Martinelli
Giovanni
G
Chilosi
Marco
M
Senna
Gianenrico
G
Pizzolo
Giovanni
G
Zanotti
Roberta
R
D000293:Adolescent; D000328:Adult; D000368:Aged; D000707:Anaphylaxis / Q000209:etiology*; D000818:Animals; D001853:Bone Marrow / Q000473:pathology*; D002648:Child; D002999:Clone Cells / Q000473:pathology; D005260:Female; D006801:Humans; D006927:Hymenoptera; D015994:Incidence; D007299:Insect Bites and Stings / Q000150:complications*; D008297:Male; D008407:Mast Cells / Q000473:pathology*; D008415:Mastocytosis / Q000201:enzymology / Q000453:epidemiology* / Q000473:pathology; D008875:Middle Aged; D017354:Point Mutation / Q000235:genetics / Q000276:immunology; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000276:immunology / Q000378:metabolism; D012882:Skin Tests; D053802:Tryptases / Q000097:blood*; D055815:Young Adult
D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases