Retrospective Evaluation of Childhood Cutaneous Mastocytosis Cases

Abstract

Aim:Mastocytosis is a rare disease characterized by clonal mast cell proliferation in one or more organs. It can lead to different clinical manifestations and has no definitive treatment. In this study, we aimed to evaluate the clinical and laboratory characteristics of our patients diagnosed 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 the Ege 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 time of 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 slightly increased. The median level of total IgE was 91.1 (4.47-362) IU/mL. Mast cell proliferation was not detected in bone marrow aspiration and biopsy 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 in selected cases.

Keywords

• mastocytosis
• cutaneous
• tryptase
• child