A case report of systemic mastocytosis associated with multiple hematologic non-mast cell lineage diseases

被引：2

作者：

Grifoni, Federica Irene ^{[1
]}

Sciume, Mariarita ^{[1
]}

Pravettoni, Valerio ^{[2
]}

Ulivieri, Fabio Massimo ^{[3
]}

Muratori, Simona ^{[4
]}

Fracchiolla, Nicola Stefano ^{[1
]}

Tagliaferri, Elena ^{[1
]}

Gianelli, Umberto ^{[5
]}

Migliorini, Anna Chiara ^{[6
]}

Cro, Lilla ^{[7
]}

Pacilli, Annalisa ^{[8
]}

Mannelli, Francesco ^{[8
]}

Baldini, Luca ^{[1
]}

机构：

[1] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Hematol Unit, Via Francesco Sforza 35, I-20122 Milan, Italy

[2] Fdn IRCCS Ca Granda Osped Maggiore Policlin, UOC Gen Med Immunol & Allergol, Milan, Italy

[3] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Bone Metab Unit, Nucl Med, Milan, Italy

[4] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dept Physiopathol & Transplantat, Dermatol Unit, Milan, Italy

[5] Univ Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, Div Pathol, Dept Pathophysiol & Transplantat, Milan, Italy

[6] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Serv Immunoematol & Trasfus, Lab Ematol, Milan, Italy

[7] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Lab Cent, Serv Citofluorimetria, Milan, Italy

[8] Univ Florence, Azienda Osped Univ Careggi, Ctr Res & Innovat Myeloproliferat Neoplasms, Dept Expt & Clin Med,CRIMM, Florence, Italy

来源：

HEMATOLOGICAL ONCOLOGY | 2019年 / 37卷 / 02期

关键词：

associated hematologic non-mast cell lineage disease; midostaurin; systemic mastocytosis; CHRONIC LYMPHOCYTIC-LEUKEMIA; ACUTE MYELOID-LEUKEMIA; MIDOSTAURIN;

D O I：

10.1002/hon.2605

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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.

引用

页码：205 / 211

页数：7

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