Author/Authors :
Ghanem, Sassine Department of Medicine - Staten Island University Hospital - Northwell Health - Staten Island - NY 10305, USA , Garcia, Gwenalyn Department of Hematology/Oncology - Staten Island University Hospital - Northwell Health - Staten Island - NY 10305, USA , Odaimi, Marcel Department of Hematology/Oncology - Staten Island University Hospital - Northwell Health - Staten Island - NY 10305, USA , Hurford, Matthew Department of Pathology - Staten Island University Hospital - Northwell Health - Staten Island - NY 10305, USA , Ying, Liu Department of Pathology - Staten Island University Hospital - Northwell Health - Staten Island - NY 10305, USA
Abstract :
Systemic mastocytosis (SM) is a disease characterized by a clonal infiltration of mast cells affecting various tissues of the body.It is grouped into six different subtypes according to the World Health Organization classification. It is called indolent systemicmastocytosis (ISM) when there is no evidence of end organ dysfunction, while the presence of end organ dysfunction definesaggressive systemic mastocytosis (ASM). When SM coexists with a clonal hematological disorder, it is classified as systemicmastocytosis with associated clonal hematological nonmast cell lineage disease (SM-AHNMD). Over 80% of SM-AHNMD casesinvolve disorders of the myeloid cell lines. To our knowledge, there are only 8 reported cases to date of SM associated witha plasma cell disorder. We report a patient with ISM who was found to have concomitant smoldering multiple myeloma. Hisdisease later progressed to ASM. We discuss this rare association between SM and a plasma cell disorder, and potential commonpathophysiologic mechanisms linking the two disorders will bereviewed.WealsodiscussprognosticfactorsinSMaswellasthemanagement options considered during the evolution of the patient’s disease.
Keywords :
Systemic Mastocytosis , Smoldering Multiple Myeloma , SM-AHNMD , ISM , Systemic mastocytosis (SM)
