Indolent systemic mastocytosis in a child: A rare and difficult diagnosis
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...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2016-01-01
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| Series: | Indian Journal of Paediatric Dermatology |
| Subjects: | |
| Online Access: | http://www.ijpd.in/article.asp?issn=2319-7250;year=2016;volume=17;issue=4;spage=306;epage=308;aulast=Torun |
| _version_ | 1818872657552080896 |
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| author | Yasemin Altuner Torun Ayse Betul Ergul Elif Guler Kazancı Mahmut Can Serbetci Fatma Mutlu Sarıguzel |
| author_facet | Yasemin Altuner Torun Ayse Betul Ergul Elif Guler Kazancı Mahmut Can Serbetci Fatma Mutlu Sarıguzel |
| author_sort | Yasemin Altuner Torun |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-19T12:42:18Z |
| format | Article |
| id | doaj.art-898a610eecbf444cbe37f7b897e60c66 |
| institution | Directory Open Access Journal |
| issn | 2319-7250 |
| language | English |
| last_indexed | 2024-12-19T12:42:18Z |
| publishDate | 2016-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Paediatric Dermatology |
| spelling | doaj.art-898a610eecbf444cbe37f7b897e60c662022-12-21T20:20:56ZengWolters Kluwer Medknow PublicationsIndian Journal of Paediatric Dermatology2319-72502016-01-0117430630810.4103/2319-7250.184333Indolent systemic mastocytosis in a child: A rare and difficult diagnosisYasemin Altuner TorunAyse Betul ErgulElif Guler KazancıMahmut Can SerbetciFatma Mutlu SarıguzelMastocytosis 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.http://www.ijpd.in/article.asp?issn=2319-7250;year=2016;volume=17;issue=4;spage=306;epage=308;aulast=TorunAnaphylaxischildhoodsystemic mastocytosis |
| spellingShingle | Yasemin Altuner Torun Ayse Betul Ergul Elif Guler Kazancı Mahmut Can Serbetci Fatma Mutlu Sarıguzel Indolent systemic mastocytosis in a child: A rare and difficult diagnosis Indian Journal of Paediatric Dermatology Anaphylaxis childhood systemic mastocytosis |
| title | Indolent systemic mastocytosis in a child: A rare and difficult diagnosis |
| title_full | Indolent systemic mastocytosis in a child: A rare and difficult diagnosis |
| title_fullStr | Indolent systemic mastocytosis in a child: A rare and difficult diagnosis |
| title_full_unstemmed | Indolent systemic mastocytosis in a child: A rare and difficult diagnosis |
| title_short | Indolent systemic mastocytosis in a child: A rare and difficult diagnosis |
| title_sort | indolent systemic mastocytosis in a child a rare and difficult diagnosis |
| topic | Anaphylaxis childhood systemic mastocytosis |
| url | http://www.ijpd.in/article.asp?issn=2319-7250;year=2016;volume=17;issue=4;spage=306;epage=308;aulast=Torun |
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