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Table 2 Summary of the investigations and demographic and clinical characteristics of patients

From: The significance of diagnosing associated clonal mast cell diseases in patients with venom-induced anaphylaxis and the role of bone marrow investigation

Case No 1 2 3
Gender Female Female Female
Current age (age with latest reaction) 72 (63) 67 (60) 71 (67)
Cause of anaphylaxis Wasp Wasp Wasp
Symptoms during manifestation(s) of anaphylaxis Syncope, hypotension Syncope, hypotension Syncope, hypotension, dyspnea
Skin prick test Vespula: Pos (2+) Vespula: Pos (3+) Vespula: Neg
Honey bee: Neg Honey bee: Neg Honey bee: Neg
ImmunoCap (normal value <0.35 kU/L) Vespula: 0.86 kE/L Vespula: 0.76 kE/L Vespula: <0.10 kE/L
Honey bee: <0.10 kE/L Honey bee: <0.10 kE/L Honey bee: <0.10 kE/L
Intracutaneous test n/a n/a Vespula: Pos (at 10-4 μg/ml concentration) Honey bee: Neg
Serum total IgE (kE/L) 14 89 35
sBT (normal value <11.4 μg/L) 12 14 23
UP-like skin lesions None None None
Multifocal mast cell clusters None None None
>25% mast cells spindle- shaped or with abnormal morphology None >25% spindle-shaped None
Abberant immunophenotype (CD2/CD25 mast cells) +/+ +/+ −/−
Kit mutation D816V + + -
Venom immunotherapy Yes, lifelong Yes, lifelong Planned for 5 years
Final diagnosis MMAS* SM# Non-clonal HVA¤
  1. SM systemic mastocytosis, MMAS monoclonal mast cell activation syndrome, UP-like urticaria pigmentosa like, HVA hymenoptera venom allergy.
  2. *MMAS diagnosis was established by fulfillment of mast cells clonality (abberant immunophenotype and/or proven c-kit mutation D816V.
  3. #SM diagnosis was established by fulfillment of 3 minor criteria (presence of atypical mast cell morphology, abberant immunophenotype and Kit mutation D816V).
  4. ¤Non-clonal HVA diagnosis was established after excluding mast cells clonality and other histopathological features of bone marrow mast cells.
  5. n/a, not analyzed; sBT Baseline serum tryptase.