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

Federica Irene Grifoni, Mariarita Sciumè, Valerio Pravettoni, Fabio Massimo Ulivieri, Simona Muratori, Nicola Stefano Fracchiolla, Elena Tagliaferri, Umberto Gianelli, Anna Chiara Migliorini, Lilla Cro, Annalisa Pacilli, Francesco Mannelli, Luca Baldini

Journal: Hematological oncology 2019;37(2):205-211

PMID: 30849188

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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.

Address: Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.; U.O.C. General Medicine-Immunology and Allergology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.; Nuclear Medicine, Bone Metabolic Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.; Dermatology Unit, Department of Physiopathology and Transplantation, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.; Division of Pathology, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.; Laboratorio di ematologia, Servizio di immunoematologia e trasfusionale, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.; Servizio di Citofluorimetria, Laboratorio Centrale, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.; Department of Experimental and Clinical Medicine, CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliera Universitaria Careggi, University of Florence, Florence, Italy.

DOI: 10.1002/hon.2605

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MeSH Terms: Aged, Amino Acid Substitution, Hematologic Neoplasms, Humans, Leukemia, Myelogenous, Chronic, BCR-ABL Positive, Male, Mastocytosis, Systemic, Mutation, Missense, Neoplasms, Second Primary, Proto-Oncogene Proteins c-kit