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

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
JournalHematological Oncology
DOIs
Publication statusPublished - Jan 1 2019

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Systemic Mastocytosis
Cell Lineage
Hematologic Neoplasms
Mast-Cell Leukemia
Leukemia, Myelomonocytic, Chronic
Mast Cells
Mutation
Therapeutics

Keywords

  • associated hematologic non–mast cell lineage disease
  • midostaurin
  • systemic mastocytosis

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

Cite this

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title = "A case report of systemic mastocytosis associated with multiple hematologic non–mast cell lineage diseases",
abstract = "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.",
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author = "Grifoni, {Federica Irene} and Mariarita Scium{\`e} and Valerio Pravettoni and Ulivieri, {Fabio Massimo} and Simona Muratori and Fracchiolla, {Nicola Stefano} and Elena Tagliaferri and Umberto Gianelli and Migliorini, {Anna Chiara} and Lilla Cro and Annalisa Pacilli and Francesco Mannelli and Luca Baldini",
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AU - Grifoni, Federica Irene

AU - Sciumè, Mariarita

AU - Pravettoni, Valerio

AU - Ulivieri, Fabio Massimo

AU - Muratori, Simona

AU - Fracchiolla, Nicola Stefano

AU - Tagliaferri, Elena

AU - Gianelli, Umberto

AU - Migliorini, Anna Chiara

AU - Cro, Lilla

AU - Pacilli, Annalisa

AU - Mannelli, Francesco

AU - Baldini, Luca

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

KW - associated hematologic non–mast cell lineage disease

KW - midostaurin

KW - systemic mastocytosis

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