title: Response to cladribine in patient with systemic mastocytosis.
abstract: BACKGROUND
  Systemic mastocytosis is a heterogeneous group of hematological disorders characterized by accumulation of mast cells in different organs.
  
  
  METHODS
  A 41-year-old woman presented with a three-year history of fatigue, occasional diarrhea, mild fever, skin rash and splenomegaly. Laboratory results showed severe anemia and thrombocytopenia. Cytological and histological investigation of bone marrow showed a marked increase of mast cells infiltration with following immunophenotype: CD117+, CD68+, CD34-, MPO-, CD15-. She was treated with cladribine 0.15 mg/kg body weight from day 1 to day 5, a total of six cycles, and achieved a good partial response, transfusion independency and normalization of spleen size. Although the patient responded to the treatment, the relapse with splenomegaly and bicytopenia was observed after 10 months.
  
  
  CONCLUSIONS
  Cladribine therapy was efficient in the patient' with systemic mastocytosis but the response was transient, so there is the need to search for new therapeutic options and more effective strategies in the treatment of patients with aggressive mast cell disorders.
authors: Clinical Center Dr. Dragiia Milovik, Clinic of Internal Medicine, Belgrade, Serbia. radojkov@eunet.rs
authors: Milica
authors: M
authors: Radojković
authors: Slobodan
authors: S
authors: Ristić
authors: Natasa
authors: N
authors: Colović
authors: Tatjana
authors: T
authors: Terzić
authors: Milica
authors: M
authors: Colović
mesh_terms: D000328:Adult; D017338:Cladribine / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D034721:Mastocytosis, Systemic / Q000188:drug therapy* / Q000473:pathology; D012074:Remission Induction
chemical_list: D007166:Immunosuppressive Agents; D017338:Cladribine