Patients with bone marrow mast cell infiltration who do not meet World Health Organization (WHO) criteria for systemic mastocytosis (SM) can still experience symptoms and are at risk of developing osteoporosis, according to a study recently published in eClinicalMedicine.
“The diagnosis of monoclonal mast cell with clinical significance MMCS requires the physician to implement specific preventive measures (as in mastocytosis), including increased duration of desensitization to hymenoptera venom, precautionary measures for anesthetic risk, and osteoporosis prevention,” study authors said.
What is SM?
Systemic mastocytosis (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.
The authors aimed to determine the clinical characteristics and risks of patients with MMCS and compare it with data from patients with a confirmed SM diagnosis. The study included 51 patients with MMCS.
Read more about SM signs and symptoms
Results showed that, on average, patients with MMCS were older than patients with SM, but that there was no significant difference in comorbidities between the two groups. More than 50% of patients with MMCS experienced allergy symptoms, including some cases of mast cell activation syndrome (MCA).
Compared to patients with SM, patients with MMCS presented fewer symptoms and were less likely to present gastrointestinal, musculoskeletal, neurological or fatigue symptoms. But dermatological symptoms were present in both groups in similar proportions. Notably, anaphylaxis and osteoporosis were more frequent in patients with MMCS.
Patients with MMCS were less likely to receive antihistamine and montelukast treatment than patients with SM. During a four-year follow-up period, none of the patients progressed to advanced SM. But most of them did not receive appropriate follow-up, the authors said.
“In conclusion, MMCS is a disorder characterized by MC with signs of clonality in BM without fulfilling SM and MMAS criteria. It may result in osteoporosis and life-threatening complications, such as severe anaphylaxis,” the authors said.
A significant number of patients with abnormal mast cell proliferation do not meet the SM criteria. But these patients can still experience symptoms and can be difficult to diagnose because of the scarce bone marrow infiltration. Mast cell degranulation symptoms can significantly affect a patient’s quality of life and require close attention and treatment.
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