Diagnosis of Mastocytosis in Children and Adults in Daily Clinical Practice.
Mastocytosis comprises a heterogeneous group of disorders characterized by clonal, neoplastic proliferation of mast cells accumulating in one or multiple organs. In the majority of cases skin involvement is the first clinical manifestation of the disease. Clinical work-up consists of a combination of morphological, immunohistochemical, flow cytometric immunophenotyping and molecular examination. Cutaneous mastocytosis predominates in children, whereas systemic mastocytosis is the most common form of the disease in adults. Therefore, different diagnostic algorithms have to be applied in adult patients and children with suspected mastocytosis. This comprehensive review presents currently defined variants of the disease and recommendations to facilitate diagnostic work-up in children and adults with suspected mastocytosis in daily clinical practice.
Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Magdalena
M
Lange
Hanna
H
Ługowska-Umer
Marek
M
Niedoszytko
Bartosz
B
Wasąg
Janusz
J
Limon
Anton
A
Żawrocki
Bogusław
B
Nedoszytko
Michał
M
Sobjanek
Katarzyna
K
Plata-Nazar
Roman
R
Nowicki
D000328:Adult; D017668:Age of Onset; D002648:Child; D003937:Diagnosis, Differential; D006801:Humans; D034701:Mastocytosis, Cutaneous / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D034721:Mastocytosis, Systemic / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D011237:Predictive Value of Tests; D011379:Prognosis