Pleural effusion as the main manifestation of intrathoracic extramedullary heamatopoietic masses in a patient with beta thalassemia minor
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Pulmonary Clinic, Aristotle University of Thessaloniki, “G. Papanikolaou” Hospital, Exohi, Thessaloniki, Greece
Konstantinos Porpodis   

Pneumonological Clinic Aristotle University of Thessaloniki, “G. Papanikolaou” Hospital, Exohi, Thessaloniki 57010, Greece
Pneumon 2016;29(2):156–160
We report a case of a patient who was admitted to the emergency Department with gradually worsening chest pain followed by shortness of breath. Chest X-ray showed right sided pleural effusion and a paravertebral lesion. Computed tomography of the chest showed sizable pleural effusion with atelectasis of the underlying lung and three intrathoracic paravertebral masses with sharp margins. Computed tomography guided fine needle biopsy was performed and established diagnosis of extramedullary haematopoiesis. The only therapeutic intervention applied was pleural fluid aspiration without relapse of the effusion during the period of ten months follow up.
Fraser R, Pare J, Fraser R, et al. The normal chest. In: Fraser R, Pare J, Fraser R et al, eds. Synopsis of diseases of the chest, 2nd ed. Philadelphia: WB Saunders 1994; 1–116.
Duwe VB, Sterman HD, Musani IA. Tumors of the Mediastinum. Chest. 2005; 128:2893-909.
Strollo DC, Rosado-de-christenson ML, Jett JR. Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum. Chest. 1997; 112:1344-57.
Glazer HS, Molina PL, Siegel MJ et-al. High-attenuation mediastinal masses on unenhanced CT. AJR Am J Roentgenol. 1991; 156:45-50.
Rivella S. The role of ineffective erythropoiesis in non-transfusiondependent thalassemia. Blood Rev 2012; 265:512-5.
Bobylev D, Zhang R, Haverich A, Krueger M. Extramedullary haematopoiesis presented as intrathoracic tumor in a patient with alpha-thalassaemia. J Cardiothorac Surg. 2013; 8:120.
Bartlett RP, Greipp PR, Tefferi A, Cupps RE, Mullan BP, Trastek VF: Extramedullary haematopoiesis manifesting as a symptomatic pleural effusion. Mayo Clin Proc 1995, 70:1161–4.
Smith P, Manjoney D, Teicher J, Choi KN, Braverman AS. Massive haemothorax due to intrathoracic extramedullary haematopoiesis in a patient with thalassemia intermedia. Chest 1974, 65:210–2.
Sohawon D, Lau KK, Lau T, Bowden DK. Extra-medullary haematopoiesis: A pictorial review of its typical and atypical locations. J Med Imaging Radiat Oncol 2012; 56:538-44.
Haidar R, Mhaidli H, Taher AT. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia. Eur Spine J 2010; 19:871–8.
Al-Marzooq YM, Al-Bahrani AT, Chopra R, Al-Momatten MI. Fineneedle aspiration biopsy diagnosis of intrathoracic extramedullary hematopoiesis presenting as a posterior mediastinal tumor in a patient with sickle cell disease: case report. Diagnostic Cytopathology 2004; 30:119-21.
Orphanidou-Vlachou E, Tziakouri-Shiakalli C, Georgiades C. Extramedullary hemopoiesis. Semin Ultrasound CT MR 2014; 35:255-62.
Taher A, Vichinsky E, Musallam K, et al. Extramedullary hematopoiesis: Guidelines for the management of non transfusion dependent thalassaemia: Thalassaemia International Federation; 2013.
Dore F, Cianciulli P, Rovasio S, et al. Incidence and clinical study of ectopic erythropoiesis in adult patients with thalassemia intermedia. Ann Ital Med Int. 1992; 7:137–40.
Shin KH, Sharma S, Gregoritch SJ, Lifeso RM, Bettigole R, Yoon SS. Combined radiotherapeutic and surgical management of a spinal cord compression by extramedullary hematopoiesis in a patient with hemoglobin E beta-thalassemia. Acta Haematol 1994; 91:154–7.
Lalwani P, Chawla R, Kumar M, Tomar AS, Raman P. Posterior mediastinal mass: Do we need to worry much?. Ann Card Anaesth 2013; 16:289-92.
Parsa K, Oreizy A. Nonsurgical approach to paraparesis due to extramedullary hematopoiesis. Report of two cases. J Neurosurg 1995; 82:657–60.
Richter E. Extramedullary hematopoiesis with intraspinal extension in thalassemia. Aktuelle Radiol 1993; 3:320–2.
Dore F, Pardini S, Gaviano E, et al. Recurrence of spinal cord compression from extramedullary hematopoiesis in thalassemia intermedia treated with low doses of radiotherapy. Am J Hematol 1993; 44:148.
Salehi SA, Koski T, Ondra SL. Spinal cord compression in betathalassemia: case report and review of the literature. Spinal Cord 2004; 42:117–23.
Haidar R, Mhaidli H, Musallam KM, Taher AT. The spine in betathalassemia syndromes. Spine (Phila Pa 1976) 2012; 37:334–9.
Ibabao J, Kassapidis S, Demetis S, et al. Bilateral pleural effusions in a thalassemia intermedia patient with posterior mediastinal extramedullary hematopoietic masses. Hemoglobin 1999; 23:249–53.
Peng MJ, Kuo HT, Chang MC. A case of intrathoracic extramedullary hematopoiesis with massive pleural effusion: successful pleurodesis with intrapleural minocycline. J Formos Med Assoc 1994; 93:445–7.
Taher A, Skouri H, Jaber W, Kanj N. Extramedullary hematopoiesis in a patient with beta-thalassemia intermedia manifesting as symptomatic pleural effusion. Hemoglobin 2001; 25:363–8.
Kupferschmid J, Shahian D, Villanueva A. Masssive hemithorax associated with intrathoracic extramedullary hematopoiesis involving the pleura. Chest 1993; 103:974–5.
Jowitt S, Burke D, Leggat H, Lewis P, Cryer R. Pleural effusion secondary to extramedullary haemopoiesis in a patient with idiopathic myelofibrosis responding to pleurodesis and hydroxyurea. Clin Lab Haem 1997; 19:283–5.
Oren I, Goldman A, Hadda N, Azzam Z, Krivoy N, Alroy G. Ascites and pleural effusion secondary to extramedullary hematopoiesis. Am J Med Sci 1999; 318:286–8.
Aessopos A, Tassiopoulos S, Farmakis D, et al. Extramedullary Hematopoiesis-Related Pleural Effusion: The Case of ß-Thalassemia. Ann Thorac Surg 2006; 81:2037-43.
Fetell MR, Stein BM. Spinal tumors. In: Rowland LP, editor. Merritt’s textbook of neurology. 8. Philadelphia: Lea & Febiger; 1989:350–64.
Hassoun H, Lawn-Tsao L, Langevin ER, Lathi ES, Palek J. Spinal cord compression secondary to extramedullary hematopoiesis: a noninvasive management based on MRI. Am J Hematol. 1991; 37:201–3.
Chaljub G, Guinto F, Crow W, Kumar R. MRI diagnosis of spinal cord compression in beta thalassemia. Spine. 1991; 16:583–4.
Klippel N, Dehou MF, Bourgain C, Schots R, Keyser J, Ebinger G. Progressive paraparesis due to thoracic extramedullary hematopoiesis in myelofibrosis. Case report. J Neurosurg. 1993; 79:125–7.
Chu KA, Lai RS, Lee CH, Lu JY, Chang HC, Chiang HT. Intrathoracic extramedullary haematopoiesis complicated by massive haemothorax in alpha-thalassaemia. Thorax 1999; 54:466–8.
Santini M, Fiorelli A, Vicidomini G, Napolitano F. Intrathoracic Extramedullary Haematopoiesis Manifested as a Neoplastic Lesion Within Anterior Mediastinum. Ann Thorac Surg 2009; 88:2001-4.
Verani R, Olson J, Moake JL: Intrathoracic extramedullary haematopoiesis: report of a case in a patient with sickle cell disease-B Thalassemia. Am J Clin Pathol 1980, 73:133–7.
Coşkun E, Süzer T, Sermez Y, Kildaci T, Tahta K: Spinal cord compression secondary to extramedullary haematopoiesis in thalassemia intermedia. Eur Spine J 1998, 7:501–4.
Smith P, Manjoney D, Teitcher J, Choi K, Braverman A. Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with thalassemia intermedia. Chest 1988; 94:658–60.
Muthuswamy PP, Shah P, Patel R, et al. Intrathoracic extramedullary hematopoiesis simulating post-traumatic intrathoracic hemorrhage. Am J Med 1989;86:358–60.
Xiros N, Economopoulos T, Papageorgiou E, Barker DL. Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with hereditary spherocytosis. Ann Hematol 2001; 80:38–40.