A Case Report of Extramedullary Hematopoiesis in Sphenoidal Sinus in Patient with Intermediate Beta Thalassemia and Review of Literature

Document Type : Case report


Assistant Professor of Hematology and Onculogy, Emam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, Iran


Extramedullary hematopoiesis is an important side effect of intermediate thalassemia. It is usually observed in paravertebral and other soft tissues as a compressive mass and rarely in intracranial, but it has not been roported in sphenoidal sinus in literature until now.
Case report
In this article a 21- year old patient with intermediate thalassemia and sphenoidal mass has been reported. The patient underwent splenectomy and cholecystectomy simultaneously, for the billiary stones. After splenectomy his erythroblast increasesd progressively in peripheral blood, and one year later he was treated, because of acute sinusitis. Imaging studies and biopsy revealed the hematopoietic mass in sphenoidal sinus. The patient did not respond to surgical and medical treatments; therefore, he was referred for the hematopoietic stem cell transplantation.
Ectopic hematopoiesis is a rare event in thalassemia, but it can appear in such important areas such as paravertebral and intracranial, which causes severe side effects. Therefore, immediate diagnosis and treatment in high risk patients can prevent these effects.


1- Aessopos A, Kati M, Meletis J. Thalassemia intermedia today: Should patients regularly receve transfusion?
Transfusion 2007; 47:792-800.
2- Borgan-Pignatti C, Galandl R. The thalassemias and related disorders, tbalassemia intermedia. 11th ed .Wintrob's
clinical hematology. 2004. Vol. 1.p.1350-52.
3- Forget, G. Thalassemia syndrome, Hematology .In: Hoffman R, Benz EJ Jr, Shattil SJ. Ed. Basic Principles and
Practice. 4rd ed. Churchill Livingstone: New York; 2005.p.574-575.
4- Wong Y, Chen F, Tai KS, Yip LKC, Tsang KWT, Chan FL, et al. Imaging features of focal intrahepatic
extramedullary haematopoiesis Br J Radiol 1999; 72 :p.906-910. Cited19times.
5- Cameron WR, Ronnert M, Brun A. Extramedullary hemopopoiesis of CNC in ostpolycythemic myeloid metaplasia.
N Engl J Med 1981; 305: 765.
6- Gologau R, lupescn I. Thoracic spinal cord compression secondary to extramedullary hematopoiesis in thelassemia
intermedia successfully treated by local radiotherapy and hydroxyurea: a case report and review of the literature. Haema
2005; 8:667-674.
7- Tsitouridis J, Stamos S, Hassapopoulou E, Tsitouridis K, Nikolopoulos P. Extramedullary paraspinal hemetopoiesis
in thelassemia: CT and MRI evaluation. Eur J Radiol 1999; 30: 33-38.
8- Cianciulli P, Sorrentino F, Morino L, Massa A, Sergiacomi GL, Donato V, et al. Radiotherapy combined with
erythropoietin for the treatment of extramedullary hematopoiesis in an alloimmunized patient with thalassemia
intermedia. Ann Hematol 1996; 72:379-381. Cited 9 times.doi: 10.1007/s002770050190.
9- Fucharoen S, Siritanaratkul N, Winichagoon P, Chowthaworn J, Siriboon W, Muangsup W, et al. Hydroxyurea
increases hemoglobin F levels and improves the effectiveness erythropoiesis in β-thalassemia/hemoglobin E disease.
Blood 1996; 87:887-892.
10- Aarabi B, Haghshenas M, Rakeii V. Visual failure caused by suprasellar extramedullary hematopoiesis in beta
thalassemia: Case report. Neurosurgery 1998; 42:922-926. Cited 10 times. doi: 10.1097/00006123-199804000-00134
11- De Paula EV, Lima CSP, Arruda VR, Alberto FL, Saad STO, Costa FF.Long-term hydroxyurea therapy in betathalassaemia
patietsn. Eur J Haematol 2003; 70:151-155. Cited 11 times. doi: 10.1034/j.1600-0609.2003.00037.x
12- Dibbern JR, DA, Loevner LA, Lieberman AP, Salhany KE, Freese A, Marcotte PJ. MR of thoracic cord
compression caused by epidural extramedullary hematopoiesis in myelodysplastic syndrome. Am J Neuroradiol 1997;
18:363-366. Cited 24 times.
13- Gatto I, Terrana V, Biondi L. Compression of the spinal cord due to proliferation of bone marrow in epidural
space in a splenectomized person with Cooley's disease. Haematologica 1954; 38:61-76.
14- Atweh GF, DeSimone J, Saunthararajah Y, Fathallah H, Weinberg RS, Nagel RL, et al. Hemoglobinopathies.
Hematology / the Education Program of the American Society of Hematology. American Society of Hematology.
Education Program; 2003.p.14-39.
15- Papavasiliou C, Gouliamos A, Vlahos L, Trakadas S, Kalovidouris A, Pouliades G. CT and MRI of symptomatic
spinal involvement by extramedullary haemopoiesis. Clinical Radiology 1990; 42:91-
92. Cited23times.doi:10.1016/S0009-9260(05)82074-4.
 16- Russo D, Pileri S, Barbieri E, Bandini G, Zaccaria A, Benfenati D, et al. Spinal cord compression by
extramedullary hematopoietic tissue in a thalassemic patient: prompt effect of radiotherapy. Haematologica
1989; 74:495-498.
17-Bradai M, Abad MT, Pissard S, Lamraoui F, Skopinski L, De Montalembert M. Hydroxyurea can eliminate
transfusion requirements in children with severe β-thalassemia. Blood 2003; 102:1529-1530. Cited36times.
doi: 10.1182/blood-2003-01-0117.
18- Wang M, Tang DC, Liu W, Chin K, Zhu JG, Fibach E, et al. Hydroxyurea exerts bi-modal dose-dependent effects
on erythropoiesis in human cultured erythroid cells via distinct pathways Br J Haematol 2002 ; 119 :1098-
1105. Cited14times. doi: 10.1046/j.1365-2141.2002.03958.x.
19- Karimi M, Drazi H, Yavarian M. Hematologic and clinical responses of thalassemia intermedia patients to
Hydroxyurea during 6 years of therapy in Iran. J pediatr Hematol Oncol 2005; 27:380-385.