Title: Haemothorax in Thalassaemia May be a Complication of Rupture of Intrathoracic Extramedullary Haematopoiesis
Authors: Anirban Chatterjee, Suman Sarkar, Amrita Roy
DOI: http://dx.doi.org/10.18535/jmscr/v3i9.04
Intrathoracic extramedullary hematopoiesis is reported in the literature but rare; typically found in the chronic hemolytic anemia. We report a beta-thalassaemia major patient, 11-y-girl, presented with hemothorax. Computed tomographic scan of the thorax showed many paravertebral and extra-pulmonary tumors. Biopsy revealed extramedullary haematopoiesis. Hemothorax in beta thalssemia should be suspicion about Intrathoracic extramedullary hematopoiesis because surgical intervention is first-line management. Keywords: extramedullary haematopoiesis; haemothorax; thalassaemia 1. Bartlet RP, Greipp PR, Tefferi A, Cupps RE, Mullan BP, Trastek VF Extramedullary hematopoiesis manifesting as asymptomatic pleural effusion. Mayo Clin Proc.1995 70:1161-1164 2. Carr ME, Todd WM Extramedullary blast crisis and hemolytic anemia in a patient with t(3p+;4q-) and rapidly evolving myelogenous leukemia South Med.1989. J 82: 762-765 3. Aessopos A , Tassiopoulos S, Farmakis D, et al .Extramedullary Hematopoiesis-Related Pleural Effusion: The Case ofß-Thalassemia. Ann Thorac Surg.2006;81:2037-2043 4. Pornsuriyasak P, Suwatanapongched T , Wangsuppasawad N et al Massive Hemothorax in a Beta-Thalassemic Patient Due toSpontaneous Rupture of Extramedullary Hematopoietic Masses:Diagnosis and Successful Treatment.Respir Care 2006;51:272–276. 5. Hsu FI, Filippa DA, Castro-Malaspina H, Downey RJ Extramedullary hematopoiesis mimicking metastatic lung carcinoma Ann Thoracic Surgery 1998.66: 1411-1413 6. Jackson A, Burton IE, Retroperitoneal mass and spinal cord compression due to extramedullary hematopoiesis in polycythemuia rubra vera. Br J Radiol.1989. 62:944-947 7. Habibzadeh F, Yadollahie M, Haghshenas M. Extra-medullary haematopoiesis in β-thalassaemia . J Postgrad Med 2005;51:45-6 8. Kupferschmidt JP. Shahian DM, Villaneuva AG Massive hemothorax associated with intrathoracic extramedullary hematopoiesis involving the pleura. Chest .1993.103:974-975 9. Pulsoni A, Ferazza G, Malagnino F, Maurillo L, Pescarmona E, Picardi A, Rendina EA, Amandori S Mediastinal extramedullary hematopoiesis as first manifestation of hereditary spherocytosis Ann Hamastol,1992. 65: 196-198 10. Smith PR, Manjoney DL, Teitcher JB, Choi KN, Braverman AS Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with thalassemia intemedia Chest.1988 94:658-660 11. Saxon BR. Rees D, Olivieri NF Regression of extramedullary hematopoiesis and augmentation of fetal hemoglobin concentration during hyrdoxyurea therapy in β thalassemia Br J Hematol.1998.101:416-419Abstract
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