Dergiler / The Journal of Pediatric Research / 2020 / Cilt: 7 - Sayı: 1

Retrospective Evaluation of Childhood Cutaneous Mastocytosis Cases

Sayfa
13–17
DOI
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Özet

Aim: Mastocytosis is a rare disease characterized by clonal mast cell proliferation in one or more organs. It can lead to different clinicalmanifestations and has no definitive treatment. In this study, we aimed to evaluate the clinical and laboratory characteristics of our patientsdiagnosed with mastocytosis in our clinic.Materials and Methods: Thirteen patients that were followed up with the diagnosis of mastocytosis at the Pediatric Hematology Clinic, in theEge University Faculty of Medicine between November 1999 and April 2016 were retrospectively analyzed.Results: Seven of patients were female (53.8%) and six were male (42.6%). The mean age at diagnosis was 20 (3-68) months. At the timeof diagnosis, complete blood count and peripheral smear were found to be compatible with the anemia of iron deficiency in three patients.Other parameters were normal. Mean tryptase level was detected as 5.9 (3.6-16.6) ng/mL, and only one tryptase level was found as slightlyincreased. The median level of total IgE was 91.1 (4.47-362) IU/mL. Mast cell proliferation was not detected in bone marrow aspiration andbiopsy material of any patients. All of the cases were evaluated as cutaneous type mastocytosis.Conclusion: The possibility of mastocytosis in systemic form in childhood is very rare and bone marrow examination may be necessary inselected cases.