Systemic mastocytosis (SM) is a rare clonal neoplastic disease in humans derived from hematopioetic stem cells. This illness presents either as cutaneous mastocytosis with benign behavior and normal life-expectancy or as SM with variable prognosis. Eminently, mast cell leukaemia (MCL) remains life-threatening with a very poor prognosis up to date. In indolent SM, clinical symptoms elicited by mast cell released mediators (among others: histamine and heparin) are often predominant resulting in a clinical picture that is similar to an anaphylactic reaction. Thus, standard therapy consists of histamine blockers and strict avoidance of symptom-triggering substances. Conversely, aggressive SM and MCL have to be treated with interferon-alpha, cladribine, KIT-tyrosine kinase inhibitors and chemotherapy. The underlying molecular and genetic background of SM is partially known and a close resemblance to canine mast cell neoplasms has been detected, but other assignable causes still remain unclear. Therefore, the Medical University and the Veterinary University of Vienna have launched a joint research initiative to explore molecular and genetic causes more precisely, thereby improving therapeutic options for both humans and dogs.
