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2017 ; 34
(4
): 306-312
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The role of serum tryptase in the diagnosis and monitoring of pediatric
mastocytosis: a single-center experience
#MMPMID28951704
Lange M
; Zawadzka A
; Schrörs S
; S?omka J
; ?ugowska-Umer H
; Nedoszytko B
; Nowicki R
Postepy Dermatol Alergol
2017[Aug]; 34
(4
): 306-312
PMID28951704
show ga
INTRODUCTION: In children, cutaneous mastocytosis (CM) is considered to be a
benign disease associated with mast cell mediator-related symptoms. However,
systemic mastocytosis (SM) and anaphylaxis may also occur. Since the basal serum
tryptase (bsT) level reflects mast cell burden, its determination is recommended
in the diagnosis and follow-up. AIM: To compare clinical presentation and the
course of mastocytosis in children with normal and clearly elevated bsT levels as
well as to assess its usefulness in the diagnosis and monitoring of the course of
the disease. MATERIAL AND METHODS: A retrospective analysis of 102 medical
records of children with mastocytosis diagnosed and followed-up in the Gdansk
Mastocytosis Center in 2014 was performed. RESULTS: Maculopapular CM (MPCM) was
diagnosed in 91 (89.22%) children, diffuse cutaneous mastocytosis (DCM) in 7
(6.86%) and mastocytoma in 4 (3.92%). The presence of flushing and bullous
lesions was more frequent in children with bsT levels > 20 ng/ml in comparison
with those who had bsT ? 20 ng/ml (p = 0.002 and p = 0.03, respectively).
Anaphylaxis occurred in 2 MPCM children with bsT levels in normal ranges. In all
of the 3 children with persistently and clearly elevated bsT levels, bone marrow
biopsy revealed no mast cells infiltrates corresponding to SM. CONCLUSIONS:
Although mastocytosis children with clearly elevated bsT levels frequently
develop mediator-related symptoms, the occurrence of anaphylaxis in this age
group may be difficult to predict. In pediatric cases with suspected SM, the bsT
level is one of the crucial parameters considered before the decision on bone
marrow biopsy.