Intracranial Hematopoiesis in Beta Thalassemia: A Case Series


Extramedullary hematopoiesis (EMH) is a normal response to failure of hematopoiesis at its normal site i.e., bone marrow. It is a manifestation of many congenital hemolytic anemias and marrow failure secondary to myelodysplastic syndromes. Usually, extramedullary myeloid proliferation occurs in liver, spleen and lymph nodes. However, there are many unusual sites where EMH can occur. The authors report two cases of intracranial extramedullary hematopoiesis in beta thalassemia. In one of these patients, epidural soft tissue was detected along frontal and parietal convexities causing compression of brain parenchyma leading to raised intracranial tension and sagging of brain stem, corpus callosum and herniation of cerebellar tonsils. The other case had a similar but unilateral epidural soft tissue. Expansion of diploic spaces of skull was seen in both these cases. As myeloid proliferation is slow, it presents with subtle symptoms of headache and gradually progressive lower limb weakness. A high index of clinical suspicion coupled with imaging findings is the only way to confirm the diagnosis.

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  1. 1.

    Origa R. Beta-thalassemia. GeneReviews(®). Seattle: University of Washington; 1993.

  2. 2.

    Taher A, Vichinsky E, Musallam K, Cappellini MD, Viprakasit V, Weatherall SD. Guidelines for the management of non transfusion dependent thalassaemia (NTDT). Nicosia, Cyprus: Thalassaemia International Federation; 2013. p. 84–91.

    Google Scholar 

  3. 3.

    Ginzel AW, Kransdorf MJ, Peterson JJ, Garner HW, Murphey MD. Mass-like extramedullary hematopoiesis: imaging features. Skelet Radiol. 2012;41:911–6.

    Article  Google Scholar 

  4. 4.

    Zherebitskiy V, Morales C, Del Bigio MR. Extramedullary hematopoiesis involving the central nervous system and surrounding structures. Hum Pathol. 2011;42:1524–30.

    Article  PubMed  Google Scholar 

  5. 5.

    Rajiah P, Hayashi R, Bauer TW, Sundaram M. Extramedullary hematopoiesis in unusual locations in hematologically compromised and noncompromised patients. Skelet Radiol. 2011;40:947–53.

    Article  Google Scholar 

  6. 6.

    Sauer B, Buy X, Gangi A, Roy C. Exceptional localization of extramedullary hematopoiesis: presacral and periureteral masses. Acta Radiol. 2007;48:246–8.

    Article  PubMed  CAS  Google Scholar 

  7. 7.

    Smithson LV, Lipper MH, Hall JA. Paranasal sinus involvement in thalassemia major: CT demonstration. AJNR Am J Neuroradiol. 1987;8:564–5.

    PubMed  CAS  Google Scholar 

  8. 8.

    Lee CM, Salzman KL, Blumenthal DT, Gaffney DK. Intracranial extramedullary hematopoiesis: brief review of response to radiation therapy. Am J Hematol. 2005;78:151–2.

    Article  PubMed  Google Scholar 

  9. 9.

    Kittner T, Aust D, Illmer T. Intracranial haemopoiesis. Lancet. 2003;362:2065.

    Article  PubMed  Google Scholar 

  10. 10.

    Kim C. Homeostatic and pathogenic extramedullary hematopoiesis. J Blood Med. 2010;1:13–9.

    Article  PubMed  PubMed Central  Google Scholar 

  11. 11.

    Koch CA, Li C-Y, Mesa RA, Tefferi A. Nonhepatosplenic extramedullary hematopoiesis: associated diseases, pathology, clinical course, and treatment. Mayo Clin Proc. 2003;78:1223–33.

    Article  PubMed  Google Scholar 

  12. 12.

    Karki B, Y-K X, Tamrakar K, et al. Intracranial extramedullary hematopoiesis in beta-thalassemia. Korean J Radiol. 2012;13:240.

    Article  PubMed  PubMed Central  Google Scholar 

  13. 13.

    Koch BL, Bisset GS, Bisset RR, Zimmer MB. Intracranial extramedullary hematopoiesis: MR findings with pathologic correlation. Am J Roentgenol. 1994;162:1419–20.

    Article  CAS  Google Scholar 

  14. 14.

    Karimi M, Cohan N, Pishdad P. Hydroxyurea as a first-line treatment of extramedullary hematopoiesis in patients with beta thalassemia: four case reports. Hematology. 2015;20:53–7.

    Article  PubMed  CAS  Google Scholar 

  15. 15.

    Taher A, Isma’eel H, Cappellini MD. Thalassemia intermedia: revisited. Blood Cells Mol Dis. 2006;37:12–20.

  16. 16.

    Emamhadi M, Alizadeh A. Effect of hypertransfusion on extramedullary hematopoietic compression mass in thalassemia major: a case report. Iran J Radiol. 2012;9:154–6.

    Article  PubMed  PubMed Central  Google Scholar 

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Correspondence to Amitkumar J. Choudhari.

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Merchant, R., Choudhari, A.J., Verma, M. et al. Intracranial Hematopoiesis in Beta Thalassemia: A Case Series. Indian J Pediatr 85, 679–681 (2018).

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  • Thalassemia
  • Intracranial extramedullary hematopoiesis
  • MRI