Systemic Mastocytosis: Clinical Update and Future Directions

Anaphylaxis as a Manifestation of Systemic Mastocytosis: A Case Report and Review of Literature

“…Fatigue is the most common symptom of SM, but other constitutional symptoms such as weight loss, anorexia, night sweats, and fevers can be presenting symptom due to cytokines secretions [15]. Increased MC mediator release also can have effects on the musculoskeletal system, with 31% of SM patients experiencing symptomology that can resemble fibromyalgia [22].…”

“…Clinically, symptoms depend on the subtype of SM and are due to MC mediator release and/or organ infiltration [15]. Skin manifestations are the hallmark of CM but also are a common manifestation of SM, especially ISM.…”

“…Elevations above 20 ng/mL in suspected patients often yields a diagnosis of SM upon full investigation [15].…”

“…KIT (CD117) is a Type III receptor tyrosine kinase that is expressed by MC, hematopoietic progenitor cells, germ cells, melanocytes and interstitial cells of Cajal in the gastrointestinal tract and is therefore functionally relevant for normal MC development, hematopoiesis, gametogenesis, melanogenesis, and regulation of slow gastric waves [16]. Overall, uncontrolled activation of the KIT receptor leads to increased production of MC and the accumulation of MC in extracutaneous organs, which can result in organ failure and even early death [15].…”

“…During her hospital stay, she presented a body flush at 96 hours with hypoxia that were attributed to the quinolones. She was put on IV ceftriaxone 2 g once daily with rapid many clinicians are not readily accustomed to diagnosing and treating SM [15].…”