Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Systemic mastocytosis (SM) is defined as the accumulation of abnormal mast cells (MC) in 1 or more extracutaneous tissues. Symptoms are due to either MC activation or organ infiltration and vary depending on disease subtype. More benign forms of SM, such as indolent SM, result in a life expectancy similar to the general population, while more aggressive subtypes, such as MC leukemia (MCL), have a median survival measured on the order of months. Treatment of indolent SM is directed at controlling the symptoms associated with MC activation. In advanced forms, such as aggressive SM and MCL, agents targeting MC proliferation such as KIT tyrosine kinase inhibitors, cladribine, and thalidomide may be provided. Newer agents based on preclinical rationale are also being actively investigated. However, the only potentially curative therapy for aggressive SM/MCL remains hematopoietic stem cell transplantation. Given that SM is a relatively rare disease, clinicians are often underprepared to evaluate, diagnose, and effectively treat this clinically heterogeneous condition. Here we seek to familiarize clinicians with this orphan disease and review current and future treatment approaches.
Clin Lymphoma Myeloma Leuk 2015 Dec
PMID:Systemic Mastocytosis: Clinical Update and Future Directions. 2638 91

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 systemic mastocytosis (ISM) when there is no evidence of end organ dysfunction, while the presence of end organ dysfunction defines aggressive systemic mastocytosis (ASM). When SM coexists with a clonal hematological disorder, it is classified as systemic mastocytosis with associated clonal hematological nonmast cell lineage disease (SM-AHNMD). Over 80% of SM-AHNMD cases involve disorders of the myeloid cell lines. To our knowledge, there are only 8 reported cases to date of SM associated with a plasma cell disorder. We report a patient with ISM who was found to have concomitant smoldering multiple myeloma. His disease later progressed to ASM. We discuss this rare association between SM and a plasma cell disorder, and potential common pathophysiologic mechanisms linking the two disorders will be reviewed. We also discuss prognostic factors in SM as well as the management options considered during the evolution of the patient's disease.
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PMID:Systemic Mastocytosis with Smoldering Multiple Myeloma: Report of a Case. 2729 30