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Indolent systemic mastocytosis in a child: A rare and difficult diagnosis


1 Department of Pediatric Hematology, Kayseri Training and Research Hospital, Kayseri, Turkey
2 Department of Pediatrics, Kayseri Training and Research Hospital, Kayseri, Turkey
3 Department of Pediatric Hematology, Bursa Dortcelik Children Hospital, Bursa, Turkey
4 Department of Immunology, Kayseri Training and Research Hospital, Kayseri, Turkey

Correspondence Address:
Yasemin Altuner Torun,
Department of Pediatric Hematology, Kayseri Training and Research Hospital, Emel Mehmet Tarman Children Hospital, Kayseri
Turkey
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.184333

Mastocytosis is a sporadic disease characterized by an abnormal accumulation of mast cells (MCs) in single or multiple organs. A 5-year-old girl who was diagnosed as cutaneous mastocytosis by our Dermatology Department when she was 4 months of age was admitted to our Pediatric Hematology Department by hepatosplenomegaly. Diagnosis of systemic mastocytosis (SM) was verified by one major and one minor WHO criteria: presence of multifocal, dense aggregates of MCs in bone marrow (major criteria) and confirmed by expression of CD2, CD25, and CD117 in bone marrow (minor criteria). During follow-up, systemic anaphylaxis was determined and treated for 3 times. We here present the first report of use of interferon-α-2a, methylprednisolone, and montelukast in life-threatening SM to achieve remission, suggesting a new option for treatment of SM in childhood.


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    -  Torun YA
    -  Ergul AB
    -  Kazancı EG
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