Intracranial Extramedullary Hematopoiesis in a Patient with Intermediate B-Thalassemia

Document Type : Case Report

Authors

1 Associate Professor, Department of Pathology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.

2 Assistant Professor, Department of Neurosurgery, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.

3 Assistant Professor, Department of Pathology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.

4 Resident, Department of Pathology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.

Abstract

Background: Extramedullary hematopoiesis refers to formation of hematopoietic tissue and it is a compensatory phenomen in hematologic disease like congenital hemolytic anemia, myelofibrosis, pernicious anemia & Bone marrow infiltration secondary to malignancies. It has been reported in many sites but the most common sites are liver, spleen & lymph node.Case Report: A 34-year-old man with intermediate B-thalassemia had chronic headache and progressive loss of visual function. In CT scan a lobulated, non homogenous hyperdense mass was seen in left occipital horn with massive surrounding edema. In histologic examination the presence of hematopoietic tissue in brain parenchyma was confirmed.

Keywords


  1. Rizzo L, Greco CS, Tola E, Sardo P, Rubino A. Extramedullary hematopoiesis: unusual menin-geal and paranasal sinuses presentation in Paget disease. Case report. Radiol Med 2003; 105(4): 376-81.
  2. Lund RE, Aldridge NH. Computed tomography of intracranial extramedullary hematopoiesis. J Comput Assist Tomogr 1984; 8(4): 788-90.
  3. Ohtsubo M, Hayashi K, Fukushima T, Chiyoda S, Takahara O. Case report: intracranial ex-tramedullary haematopoiesis in postpolycy-themic myelofibrosis. Br J Radiol 1994; 67(795):
  4. -302.
  5. Boyacigil S, Ali A, Ardic S, Yuksel E. Epidural extramedullary haemopoiesis in thalassaemia. Australas Radiol 2002; 46(2): 180-2.
  6. Urman M, O'Sullivan RA, Nugent RA, Lentle BC. Intracranial extramedullary hematopoiesis. CT and bone marrow scan findings. Clin Nucl Med 1991; 16(6): 431-4.
  7. Chourmouzi D, Pistevou-Gompaki K, Plataniotis G, Skaragas G, Papadopoulos L, Drevelegas A. MRI findings of extramedullary haemopoiesis. Eur Radiol 2001; 11(9): 1803-6.
  8. Buetow PC, Perry JJ, Geyer CA. Gd-DTPA en-hancement in CNS extramedullary hematopoie-sis. AJNR Am J Neuroradiol 1990; 11(2): 411-2.
  9. Aarabi B, Haghshenas M, Rakeii V. Visual fail-ure caused by suprasellar extramedullary hema-topoiesis in beta thalassemia: case report. Neu-rosurgery 1998; 42(4): 922-5.
  10. Korsten J, Grossman H, Winchester PH, Canale VC. Extramedullary hematopoiesis in patients with thalassemia anemia. Radiology 1970; 95(2): 257-63.
  11. Le Bousse-Kerdiles MC, Martyre MC. Dual im-plication of fibrogenic cytokines in the patho-genesis of fibrosis and myeloproliferation in my-eloid metaplasia with myelofibrosis. Ann Hema-tol 1999; 78(10): 437-44.
  12. Le Bousse-Kerdiles MC, Martyre MC. Involve-ment of the fibrogenic cytokines, TGF-beta and bFGF, in the pathogenesis of idiopathic myelofi-brosis. Pathol Biol (Paris) 2001; 49(2): 153-7.
  13. Polliack A, Rosenmann E. Extramedullary hem-atopoietic tumors of the cranial dura mater. Ac-ta Haematol 1969; 41(1): 43-8.
  14. Brannan D: Extramedullary hematopoiesis in anemias.Bull Johns Hopkins Hosp 1927; 41: 104-36.
  15. Beckner ME, Lee JY, Schochet SS, Jr., Chu CT. Intracranial extramedullary hematopoiesis asso-ciated with pilocytic astrocytoma: a case report. Acta Neuropathol 2003; 106(6): 584-7
  16. Gregorios JB, Bay JW, Dudley AW, Jr. Extrame-dullary hematopoiesis in a malignant meningio-ma. Neurosurgery 1983; 13(4): 447-51.
  17. Brouland JP, Megarbane B, Kafe H, Brouet JC, Mikol J. Cerebro-meningeal localization of ex-tramedullary haematopoiesis (EMH), a rare complication of chronic idiopathic myelofibrosis (CIM). Neuropathol Appl Neurobiol 2004; 30(4): 396-401.
  18. Guermazi A, de KE, Cazals-Hatem D, Zagdanski AM, Frija J. Imaging findings in patients with myelofibrosis. Eur Radiol 1999; 9(7): 1366-75.
  19. Haidar S, Ortiz-Neira C, Shroff M, Gilday D, Blaser S. Intracranial involvement in extrame-dullary hematopoiesis: case report and review of the literature. Pediatr Radiol 2005; 35(6): 630-4.
  20. Koch BL, Bisset GS, III, Bisset RR, Zimmer MB. Intracranial extramedullary hematopoiesis: MR findings with pathologic correlation. AJR Am J Roentgenol 1994; 162(6): 1419-20.
  21. Kuhn E, Dorji T, Rodriguez J, Rosai J. Extrame-dullary erythropoiesis in chronic subdural hema-toma simulating metastatic small round cell tu-mor. Int J Surg Pathol 2007; 15(3): 288-91.