Patients with systemic mastocytosis (SM) appear to have a different plasma protein profile from healthy individuals, according to a recently published abstract to be presented at the American Society of Hematology’s annual meeting.
“The heterogenous profile of circulating plasma proteins even within patients with ISM mirrors the clinically heterogeneous nature of the disease, and further understanding of the plasma proteins within an individual patient may hold prognostic or therapeutic value,” the authors said.
What is SM?
Systemic mastocytosisMastocytosis Rare disease caused by the buildup of mast cells. Cutaneous mastocytosis primarily affects the skin and is more common in children, while systemic mastocytosis affects internal organs and is more common in adults. (SM) is a rare hematological disease characterized by mast cells that are overactive and accumulate in different parts of the body such as the bone marrow, liver, spleen, gastrointestinal tract and lymph nodes.
Researchers analyzed the plasma samples of more than 160 patients with indolent SMIndolent SM A subtype of nonadvanced SM caused by the abnormal accumulation of mast cells in the bone marrow and other organs. Indolent SM accounts for around 90% of SM cases. enrolled in the PIONEER study and compared them to samples from 39 healthy volunteers. The Olink Explore 384 Inflammation (Uppsala, Sweden) plasma protein panel detected significant differences between the blood of patients with SM and healthy controls.
Read more about SM signs and symptoms
Out of more than 300 detected proteins, 161 were differentially expressed in both groups. The search detected proteins involved in mast cellMast cell A type of white blood cell produced in the bone marrow. They help defend against infections and play a key role in allergic reactions. In SM, mast cells become overactive and build up throughout the body. degranulation and activation, such as SIGLEC10 and mast cell immunoglobulin-like receptor 1, in patients with indolent SM. Further, researchers found significant differences in proteins associated with inflammation and the immune system.
“A number of proteins have been identified in patients with ISM which could aid in future understanding of the disease and therapeutic targets for its treatment. Plasma protein analysis provides a new tool with which to understand and characterize ISM,” the authors said.
Despite the introduction of new therapies in recent decades, many patients with SM still struggle with life-altering symptoms.
Inflammation plays a key role in the pathogenesis of SM and is responsible for many of the symptoms associated with the disease. But many of the blood mediators that drive it are unknown. This is the first study that extensively studied the proteome in a large population of patients with indolent SM and compared it with that of healthy individuals.
A better understanding of the mediators involved in SM inflammation might enable investigators to detect potential therapeutic targets and develop new drugs.
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