title: [Symptomatic extramedullary haematopoiesis in β-thalassemia: A retrospective single centre study].
abstract: BACKGROUND
  Symptomatic extramedullary hematopoiesis (EH) is a rare but potentially severe phenomenon which occurs in β-thalassemia. There are no treatment guidelines.
  
  
  METHODS
  Retrospective single centre study including the cases of symptomatic EH encountered between 1997 and 2014 in a unit specialised in red blood cell genetic disorders. Description of clinical, biological and radiological characteristics of the patients, treatments received, and outcomes.
  
  
  RESULTS
  Among 182 β-thalassemia patients followed during the study period, 7 cases of symptomatic EH were diagnosed. They were 5 men and 2 women, and their mean age was 37 years. Four patients were splenectomised, two patients were regularly transfused, and four patients had already received erythropoietin. EH was localised in intravertebral areas and responsible for dorsal spinal cord compression in 5 patients, in paravertebral dorsal area in 1 patient, and in presacral area in 1 patient. The mean hemoglobin level at diagnosis was 7.9 g/dL. Treatment administered included: red cell transfusion in 6 cases, associated with hydroxyurea in 5 cases and/or radiotherapy in 3 patients. One patient was treated with surgery and HU. After a median follow-up of 41 months, clinical recovery was complete in 2 patients and partial in 5 patients.
  
  
  CONCLUSIONS
  EH must be suspected in β-thalassemia in patients presenting clinical signs of organ compression, and a typical radiological aspect. The functional prognosis depends on the rapidity of treatment, which includes red blood cell transfusion, hydroxyurea, radiotherapy, and rarely surgery. Long-term outcome is uncertain.
authors.affiliation: Unité des maladies génétiques du globule rouge, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: F
authors.initials: F
authors.lastname: Maazoun
authors.affiliation: Unité des maladies génétiques du globule rouge, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France; Service de médecine interne, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France. Electronic address: justine.gellen@gmail.com.
authors.forename: J
authors.initials: J
authors.lastname: Gellen Dautremer
authors.affiliation: Service d'imagerie médicale, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: A
authors.initials: A
authors.lastname: Boutekadjirt
authors.affiliation: Service de biochimie-génétique, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: S
authors.initials: S
authors.lastname: Pissard
authors.affiliation: Unité des maladies génétiques du globule rouge, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: A
authors.initials: A
authors.lastname: Habibi
authors.affiliation: Unité des maladies génétiques du globule rouge, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: D
authors.initials: D
authors.lastname: Bachir
authors.affiliation: Service d'imagerie médicale, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: A
authors.initials: A
authors.lastname: Rahmouni
authors.affiliation: Unité des maladies génétiques du globule rouge, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France; Service de médecine interne, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: P
authors.initials: P
authors.lastname: Bartolucci
authors.affiliation: Unité des maladies génétiques du globule rouge, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: K
authors.initials: K
authors.lastname: Debbache
authors.affiliation: Service de radiothérapie, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: J-L
authors.initials: JL
authors.lastname: Lagrange
authors.affiliation: Service de médecine interne, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: M
authors.initials: M
authors.lastname: Michel
authors.affiliation: Unité des maladies génétiques du globule rouge, hôpital Henri-Mondor, 51, avenue Maréchal-de-Lattre-de-Tassigny, 94110 Créteil cedex, France.
authors.forename: F
authors.initials: F
authors.lastname: Galacteros
mesh_terms: D000328:Adult; D005260:Female; D006411:Hematopoiesis, Extramedullary / Q000235:genetics / Q000502:physiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult; D017086:beta-Thalassemia / Q000235:genetics / Q000503:physiopathology*
keywords: Anemia; Anémie; Extramedullary hematopoiesis; Hématopoïèse extra-médullaire; β-thalassemia; β–thalassémie