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Objective To review the clinical features,diagnostic work-up,classification,prognosis and treatment of systemic mastocytosis (SM).Methods The clinical data of 3 SM patients admitted to Peking Union Medical College Hospital (PUMCH) were retrospectively analyzed and the review of recent literatures was performed.Results All the 3 cases were pathologically diagnosed.According to WHO 2008 classification criteria,2 cases were diagnosed as aggressive SM (ASM) and the other one was diagnosed as indolent SM (ISM).Case 1 was a 60-year-old female patient who had overt mediator release syndrome manifesting as episodes of flushing,fever,vomiting,palpitation,hypotension and syncope.She was diagnosed as aggressive SM based on significantly increased number of abnormal mast cells (accounting for 6％ of all nucleated cells) in the bone marrow aspiration sample and biopsy specimen which accompanied with decreased other myeloid and erythroid elements.Her disease was refractory to the treatment of H2 antihistamines and led to a fatal outcome eventually.Case 2 was a 72-year-old male patient who underwent extended proximal gastrectomy with esophagogastric anastomosis due to endoscopically detected diffuse thickening of the gastric fundic mucosa.The diagnosis as indolent SM was established according to the dense infiltrate of abnormal mast cells in the surgically resected part of stomach and regional lymph nodes.He had been in complete remission for more than 6 years ever since then.Case 3 was a 41-year-old female patient who presented with fever,urticaria pigmentosa and diffuse bone lesions.A bone biopsy specimen demonstrated a dense infiltrate of mast cells while the assays for KIT-D816V mutation and FIP1L1-PDGFRα fusion gene yielded negative results.She was diagnosed as aggressive SM and a progression-free survival of more than 1 year had been achieved with the treatment of prednisone,interferon-α and pamidronate.Conclusions SM is a rare disease.Diagnosis is primarily dependent on histopathology.There is currently no curative therapy for systemic mastocytosis.Treatment is intended to reduce symptoms and improve quality of life.The prognosis of ISM is much better than that of ASM.