title: Systemic mastocytosis: predictable factors of poor prognosis present at the onset of the disease.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Hôtel-Dieu, Lyon, France. katia.stankovic@chu-lyon.fr
authors.forename: Katia
authors.initials: K
authors.lastname: Stankovic
authors.forename: Françoise
authors.initials: F
authors.lastname: Sarrot-Reynauld
authors.forename: Marie
authors.initials: M
authors.lastname: Puget
authors.forename: Christian
authors.initials: C
authors.lastname: Massot
authors.forename: Jacques
authors.initials: J
authors.lastname: Ninet
authors.forename: Bernard
authors.initials: B
authors.lastname: Lorcerie
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Dupond
authors.forename: Gilles
authors.initials: G
authors.lastname: Salles
authors.forename: Isabelle
authors.initials: I
authors.lastname: Durieu
authors.forename: Denis
authors.initials: D
authors.lastname: Vital Durand
authors.forename: Hugues
authors.initials: H
authors.lastname: Rousset