Response to therapy with interferon alpha-2b and prednisolone in aggressive systemic mastocytosis: report of five cases and review of the literature

Systemic mastocytosis in adults: 2015 update on diagnosis, risk stratification, and management

“…IFN-a is often considered the first-line cytoreductive therapy in symptomatic SM; since the initial report in 1992 [79], several case reports or small series have shown IFN-a (IFN-a2b in most instances) to improve symptoms of MC degranulation, decrease BM MC infiltration, and ameliorate mastocytosis-related ascites/hepatosplenomegaly, cytopenias, skin findings, and osteoporosis [80][81][82][83][84][85][86][87][88][89][90][91][92]. IFN-a treatment is not uniformly effective [93], and the frequency of major response (i.e., complete resolution of one or more baseline 'C' findings) is 20-30%; the optimal dose and duration of IFN-a therapy for SM remain unclear, however concurrent administration of corticosteroids (prednisone) may improve its efficacy (up to 40% major response rate) and tolerability [87,94].…”

“…IFN-a treatment is not uniformly effective [93], and the frequency of major response (i.e., complete resolution of one or more baseline 'C' findings) is 20-30%; the optimal dose and duration of IFN-a therapy for SM remain unclear, however concurrent administration of corticosteroids (prednisone) may improve its efficacy (up to 40% major response rate) and tolerability [87,94]. The time to best response may be a year or longer [87] and delayed responses to therapy have been described [95].…”

“…IFN-a treatment is not uniformly effective [93], and the frequency of major response (i.e., complete resolution of one or more baseline 'C' findings) is 20-30%; the optimal dose and duration of IFN-a therapy for SM remain unclear, however concurrent administration of corticosteroids (prednisone) may improve its efficacy (up to 40% major response rate) and tolerability [87,94]. The time to best response may be a year or longer [87] and delayed responses to therapy have been described [95]. IFN-a treatment is frequently (up to 50%) complicated by toxicities, including flu-like symptoms, bone pain, fever, cytopenias, depression, and hypothyroidism; consequently, the adverse dropout rate with IFN-a treatment is not trivial [87,96,97].…”

“…The time to best response may be a year or longer [87] and delayed responses to therapy have been described [95]. IFN-a treatment is frequently (up to 50%) complicated by toxicities, including flu-like symptoms, bone pain, fever, cytopenias, depression, and hypothyroidism; consequently, the adverse dropout rate with IFN-a treatment is not trivial [87,96,97]. Finally, a significant proportion of patients will relapse within a short period of IFN-a treatment being discontinued, illustrating the cytostatic rather than cytolytic effects of the drug [97].…”

Systemic Mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disorder (MDS-RCMD): A Difficult Disease to Diagnose and Treat

“…In patients who progress slowly, it is possible to keep the disease under control for months to years with INF-a. Interferon is usually started at a dose of three million units thrice a week, increased to a maximum of five million units daily [2,3]. However, the toxicity (myalgia, flu-like symptoms) of this treatment is often a limiting factor.…”