A case report of mast cell leukemia in a child and literature review
نویسندگان
چکیده
Mast cell leukemia (MCL) is a very rare form of aggressive systemic mastocytosis accounting for < 1% all mastocytosis. Mastocytosis broad term used group clonal disorders characterized by accumulation mast cells in the skin with or without extracutaneous involvement. The clinical spectrum disease varies from only cutaneous lesions to highly involvement such as MCL. can present birth adulthood. In children, usually benign, and there good chance spontaneous regression at puberty, unlike adult-onset disease, which generally more severe. Individuals may be risk developing hematologic malignancies. MCL diagnosis requires presence SM criteria additional features including leukemic infiltration bone marrow and/or blood least 20% high-grade MC well organs neoplastic MC. Genetic aberrations, mainly KIT D816V mutation, play crucial role pathogenesis are detected most patients. To date, no approved standard therapy. For MCL, few options available treatment because rarity trials address question. Even if occurs occasionally, children require planned follow-up over time. We an overview literature on case diagnosed 4-year-old girl who had since early childhood. A examination revealed She ultimately died despite chemotherapy. patient's parents gave consent use their child's data, photographs, research purposes publications.
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ژورنال
عنوان ژورنال: Voprosy gematologii/onkologii i immunopatologii v pediatrii
سال: 2023
ISSN: ['1726-1708', '2414-9314']
DOI: https://doi.org/10.24287/1726-1708-2023-22-1-126-138