title: Effect of hydroxyurea on extramedullary haematopoiesis in thalassaemia intermedia: case reports and literature review.
abstract: Extramedullary haematopoiesis (EH) is the production of blood cell precursors outside the bone marrow that occurs in various disorders, such as thalassaemia, sickle cell anaemia, hereditary spherocytosis, polycythaemia vera, myelofibrosis and other haematological diseases. In chronic anaemia, it is a physiological response to increased erythropoietin. In some other conditions, such as myeloid metaplasia, polycythaemia vera or chronic myeloid leukaemia, EH is due to a clonal disorder of haematopoiesis that enables progenitor cells to escape from the marrow and lodge in other organs. EH usually involves the liver, spleen and lymph nodes or it can be paravertebral, intrathoracic, pelvic. It is often asymptomatic but can sometimes lead to symptomatic tumour-like masses. Treatment options are still controversial and limited, including hypertransfusion regimen, surgical treatment, radiotherapy and hydroxyurea (HU). We describe intrathoracic and symptomatic pelvic EH masses in a 48-year-old woman and intrathoracic bilateral masses causing respiratory insufficiency with pleural effusion in a 42-year-old male, both affected by thalassaemia intermedia. Both patients showed a clinical improvement with hydroxyurea therapy and occasional blood transfusions.
authors.affiliation: Department of Paediatrics, Policlinico G. Martino, University of Messina, Messina, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Meo
authors.forename: E
authors.initials: E
authors.lastname: Cassinerio
authors.forename: R
authors.initials: R
authors.lastname: Castelli
authors.forename: D
authors.initials: D
authors.lastname: Bignamini
authors.forename: L
authors.initials: L
authors.lastname: Perego
authors.forename: M D
authors.initials: MD
authors.lastname: Cappellini
mesh_terms: D000328:Adult; D005260:Female; D006411:Hematopoiesis, Extramedullary / Q000187:drug effects*; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D019384:Nucleic Acid Synthesis Inhibitors / Q000627:therapeutic use*; D010388:Pelvis / Q000473:pathology; D035423:Thoracic Cavity / Q000473:pathology; D017086:beta-Thalassemia / Q000150:complications / Q000188:drug therapy* / Q000473:pathology
chemical_list: D019384:Nucleic Acid Synthesis Inhibitors; D006918:Hydroxyurea