A case report of systemic mastocytosis associated with multiple hematologic non-mast cell lineage diseases.
associated hematologic non-mast cell lineage disease
midostaurin
systemic mastocytosis
Journal
Hematological oncology
ISSN: 1099-1069
Titre abrégé: Hematol Oncol
Pays: England
ID NLM: 8307268
Informations de publication
Date de publication:
Apr 2019
Apr 2019
Historique:
received:
14
10
2018
revised:
23
02
2019
accepted:
05
03
2019
pubmed:
9
3
2019
medline:
8
5
2019
entrez:
9
3
2019
Statut:
ppublish
Résumé
Systemic mastocytosis (SM) is a hematological malignancy characterized by extracutaneous infiltration by atypical mast cells. Together with indolent SM, aggressive SM, and mast cell leukemia, the World Health Organization (WHO) recognizes another major disease subgroup: SM with an associated hematological neoplasm, which is characterized by the presence of a concurrent neoplasm, more commonly, a chronic myelomonocytic leukemia. While KIT D816V is commonly regarded as the driver mutation, the clinical presentation of SM is extremely varied. Treatment of SM might not be simple, but now more specific therapies tailored toward prognostic subgroups of patients have been developed. Here, we report a detailed description of clinical management and biological features of a systemic mastocytocis case associated with multiple hematologic non-mast cell lineage diseases.
Substances chimiques
KIT protein, human
EC 2.7.10.1
Proto-Oncogene Proteins c-kit
EC 2.7.10.1
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
205-211Informations de copyright
© 2019 John Wiley & Sons, Ltd.