Systemic mastocytosis: Predictable factors of poor prognosis present at the onset of the disease

Systemic mastocytosis (SM) refers to a group of heterogeneous diseases that can be divided into indolent SM, for which prognosis is favorable, and malignant SM, which has a poor prognosis. While the diagnosis of SM is often a challenge since clinical and biological abnormalities are not specific, prognosis is even more difficult to predict. Thus, we aimed to highlight predictable factors in a cohort of 28 cases of SM. Among the 13 women and 15 men studied were 7 patients who had an aggressive form of SM that ultimately led to death in 3 of them. We found common characteristics among these seven patients. First, they were older when the first symptoms appeared and when the diagnosis was confirmed. Second, ascitis, lymphadenopathy, anemia, and thrombocytopenia were significantly more frequent, while cutaneous lesions and flush were less frequent. Moreover, general symptoms, gastrointestinal disorders, neutropenia, and coagulation abnormalities also seemed to characterize this group of patients. Understanding the factors that predict SM is essential in order to provide patients with the malignant form of the disease with specific treatments.