title: Systemic mastocytosis--a systematic review.
abstract: BACKGROUND
  The mast cell lives a hidden life, but it is implicated in several physiological reactions. Its ability to react to different stimuli impacts a variety of conditions such as asthma, atopic dermatitis, urticaria and anaphylaxis. It is not until recent decades that the evolution of the cell has been described and its fascinating biology has only recently been depicted. We here give a review of systemic mastocytosis in regards to cell biology, diagnostic approaches and clinical practice.
  
  
  METHODS
  A search was made in PubMed in August 2011 entering the keywords: mastocytosis, (systemic, cutaneous, aggressive), mast cell leukaemia, mast cell sarcoma, chromosome, mutation, haematology and treatment.
  
  
  RESULTS
  Mastocytosis is characterized by an abnormal proliferation of mast cells, which accumulate in one or several organ systems, primarily the skin and bone marrow. The disease is clinically heterogeneous and varies from a relatively benign condition with isolated cutaneous lesions to a very aggressive systemic condition with a grave prognosis. The condition affects men and women equally. Children are especially affected by the cutaneous form. In most children, the condition will improve or remit spontaneously before adulthood. Mastocytosis in adults, however, is more often systemic and tends to persist.
  
  
  CONCLUSIONS
  Patients with mastocytosis represent a heterogeneous group in terms of clinical presentation, management and prognosis. Furthermore, a range of medical specialties serve as the primary entrance to health services, which can be a challenge in respect of achieving uniform management. In order to improve diagnostics and management of systemic mastocytosis, the European Competence Network on Mastocytosis has been established. Patients under suspicion of systemic mastocytosis should be conferred with or referred to a haematological and a dermatological/allergological department.
authors.affiliation: Department of Haematology, Roskilde Hospital, Denmark. christenla@gmail.com
authors.forename: Christen Lykkegaard
authors.initials: CL
authors.lastname: Andersen
authors.forename: Thomas Kielsgaard
authors.initials: TK
authors.lastname: Kristensen
authors.forename: Marianne Tang
authors.initials: MT
authors.lastname: Severinsen
authors.forename: Michael Boe
authors.initials: MB
authors.lastname: Møller
authors.forename: Hanne
authors.initials: H
authors.lastname: Vestergaard
authors.forename: Olav J
authors.initials: OJ
authors.lastname: Bergmann
authors.forename: Hans Carl
authors.initials: HC
authors.lastname: Hasselbalch
authors.forename: Ole Weis
authors.initials: OW
authors.lastname: Bjerrum
mesh_terms: D005938:Glucocorticoids / Q000627:therapeutic use; D006633:Histamine Antagonists / Q000627:therapeutic use; D006801:Humans; D008407:Mast Cells / Q000473:pathology*; D034721:Mastocytosis, Systemic / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D011379:Prognosis
chemical_list: D005938:Glucocorticoids; D006633:Histamine Antagonists