[Symptomatic extramedullary haematopoiesis in XXXX : A retrospective single centre study].

@entity1 @entity1862 @entity1307 @entity1745 @entity74 @entity2395
INTRODUCTION: Symptomatic extramedullary hematopoiesis (EH) is a rare but potentially severe phenomenon which occurs in @entity2395 . 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 @entity74 . Description of clinical, biological and radiological characteristics of the @entity1 , treatments received, and outcomes. RESULTS: Among 182 @entity2395 @entity1 followed during the study period, 7  cases of symptomatic EH were diagnosed. They were 5  @entity1 and 2  @entity1 , and their mean age was 37  years. Four @entity1 were splenectomised, two @entity1 were regularly transfused, and four @entity1 had already received @entity1307 . EH was localised in intravertebral areas and responsible for dorsal @entity1745 in 5  @entity1 , in paravertebral dorsal area in 1  @entity1 , and in presacral area in 1  @entity1 . The mean hemoglobin level at diagnosis was 7.9  g/dL. Treatment administered included: red cell transfusion in 6 cases, associated with @entity1862 in 5 cases and/or radiotherapy in 3  @entity1 . One @entity1 was treated with surgery and HU. After a median follow-up of 41  months, clinical recovery was complete in 2  @entity1 and partial in 5  @entity1 . CONCLUSION: EH must be suspected in @entity2395 in @entity1 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, @entity1862 , radiotherapy, and rarely surgery. Long-term outcome is uncertain.
