title: Extramedullary hematopoiesis-related pleural effusion: the case of beta-thalassemia.
abstract: BACKGROUND
  Thalassemia intermedia has a later clinical onset and a milder anemia than thalassemia major, characterized by high output state, left ventricle remodeling, and age-related pulmonary hypertension. Bone deformities, extramedullary hematopoiesis (EMH), and spleen and liver enlargement are the consequences of hypoxia and enhanced erythropoiesis. The EMH-related pleural effusion is rarely referred to in the literature of thalassemia.
  
  
  METHODS
  We reviewed the thalassemia patients' medical records hospitalized for pleural effusion in our Department, within the last 6 years.
  
  
  RESULTS
  Eight (4 men) thalassemia intermedia patients admitted for symptomatic pleural effusion were identified. Common clinical findings on admission were dyspnea and apyrexia. Their mean hemoglobin level was 7.15 +/- 0.64 g/dL. Radiology revealed intrathoracic EMH and pleural effusion in all patients: exudative in seven patients and massive hemothorax in one. Cytologic fluid analysis was negative for malignancy. Fluid and serum cultures, antibodies, and stains were negative for viral, bacterial, and fungal infection. The hemothorax case was successfully treated with repeated aspirations, transfusions, and hydroxyurea. Although repeated thoracentesis and radiation could not control the effusions in the rest of the cases, pleurodesis was successful in 5 patients, without serious adverse events. Treatment was further accomplished with hydroxyurea. No relapses were observed in the mean 30 month follow-up period.
  
  
  CONCLUSIONS
  Afebrile, EMH-related pleuritis represents a potentially life-threatening complication in thalassemia. Therapy should be individualized and treatment is emerging. Pleurodesis seems to be an effective and safe therapeutic option for exudative effusions, while transfusion-chelation therapy combined with hydroxyurea may be helpful in suppressing increased erythropoiesis.
authors.affiliation: First Department of Internal Medicine, School of Medicine, University of Athens, Laiko Hospital, Athens, Greece.
authors.forename: Athanassios
authors.initials: A
authors.lastname: Aessopos
authors.forename: Stergios
authors.initials: S
authors.lastname: Tassiopoulos
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Farmakis
authors.forename: Ioannis
authors.initials: I
authors.lastname: Moyssakis
authors.forename: Maria
authors.initials: M
authors.lastname: Kati
authors.forename: Katerina
authors.initials: K
authors.lastname: Polonifi
authors.forename: Maria
authors.initials: M
authors.lastname: Tsironi
mesh_terms: D000328:Adult; D001281:Atrial Fibrillation / Q000209:etiology; D001803:Blood Transfusion; D015505:Chest Tubes; D003131:Combined Modality Therapy; D004417:Dyspnea / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006411:Hematopoiesis, Extramedullary / Q000187:drug effects; D006491:Hemothorax / Q000209:etiology; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D006935:Hypercapnia / Q000209:etiology; D006976:Hypertension, Pulmonary / Q000209:etiology; D017379:Hypertrophy, Left Ventricular / Q000209:etiology; D000860:Hypoxia / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010996:Pleural Effusion / Q000188:drug therapy / Q000209:etiology* / Q000601:surgery; D018700:Pleurodesis; D012189:Retrospective Studies; D013156:Splenectomy; D017086:beta-Thalassemia / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology / Q000601:surgery / Q000628:therapy
chemical_list: D006918:Hydroxyurea