Extramedullary Hematopoiesis Presenting as Progressive Headache: A Case Report and Review of Literature
-
MohammadReza Babaee
,
Iman Mohseni
,
MohammadAli Mohammadi-Vajari
,
Ghazale Tefagh
,
Nima Rakhshankhah
,
Marzieh Motevalli
Abstract
Extramedullary Hematopoiesis (EMH) is defined as the production of blood cells in organs other than bone marrow. Intracranial EMH is a rare condition. In this article, we presented a case of intracranial EMH presenting as progressive headache. Our patient was a 33-year- old man with thalassemia presenting with acute progressive flaccid quadriplegia, severe progressive headache, and decreased level of consciousness. His imaging studies showed evidence of intracranial and presacral EMH. Most asymptomatic intracranial EMH can present as a variety of symptoms, including progressive headache; therefore, the differential diagnosis should be kept in mind when evaluating a patient with a relevant underlying medical condition.
[1] Golub R, Cumano A. Embryonic hematopoiesis. Blood Cells, Molecules, and Diseases. 2013; 51(4):226-31. [DOI:10.1016/j. bcmd.2013.08.004] [PMID]
[2] Koch BL, Bisset GS, Bisset RR, Zimmer MB. Intracranial extramedul- lary hematopoiesis: MR findings with pathologic correlation. Ameri- can Journal of Roentgenology. 1994; 162(6):1419-20. [DOI:10.2214/ ajr.162.6.8192010] [PMID]
[3] Haidar S, Ortiz-Neira C, Shroff M, Gilday D, Blaser S. Intracranial involvement in extramedullary hematopoiesis: Case report and review of the literature. Pediatric Radiology. 2005; 35(6):630-4. [DOI:10.1007/s00247-004-1361-y] [PMID]
[4] Kaya H. Cerci SS. Tc-99m nanocolloid scintigraphic imaging of in- tracranial meningeal extramedullary hematopoiesis in a patient with idiopathic myelofibrosis. Annals of Nuclear Medicine. 2006; 20(8):565-8. [DOI:10.1007/BF03026823] [PMID]
[5] Singer A, Quencer R. Intracranial extramedullary hematopoiesis: A rare cause of headaches. Journal of Neuroimaging. 2014; 24(5):524-7. [DOI:10.1111/jon.12029] [PMID]
[6] Kim CH. Homeostatic and pathogenic extramedullary hematopoie- sis. Journal of Blood Medicine. 2010; 1:13-9. [DOI:10.2147/JBM. S7224] [PMID] [PMCID]
[7] 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. Amer- ican Journal of Neuroradiology. 1997; 18(2):363-6. [PMID] [PMCID]
[8] Karki B, Xu YK, Tamrakar K, Wu YK. Intracranial extramedullary he- matopoiesis in beta-thalassemia. Korean Journal of Radiology. 2012; 13(2):240-3. [DOI:10.3348/kjr.2012.13.2.240] [PMID] [PMCID]
[9] Wang A, Carberry N, Solli E, Gillick J, Islam H, Hillard V. Spinal cord compression secondary to extramedullary hematopoiesis: Case re- port and review of the literature. Case Reports in Oncolog. 2016; 9(2):290-7. [DOI:10.1159/000446473] [PMID] [PMCID]
[10] Merchant R, Choudhari AJ, Verma M, Patkar DP, Doctor P. Intrac- ranial hematopoiesis in beta thalassemia: A case series. The Indian Journal of Pediatrics. 2018; 85(8):679-81. [DOI:10.1007/s12098- 018-2618-z] [PMID]
[11] Tayari N, Ahrar MH, Jafarpishe MS. Case report of the extramedul- lary hematopoiesis presented as a hypervascular intracranial mass. Advanced Biomedical Research. 2013; 2:34. [DOI:10.4103/2277- 9175.109719] [PMID] [PMCID]
[12] Musolino A, Guazzi A, Lazzaretti M, Pezzuolo D, Calzetti C, Degli Antoni A, et al. Intracranial hematopoiesis in a patient with AIDS- related central nervous system lymphoma and severe pancytope- nia. Haematologica. 2007; 92(5):e59-61. [DOI:10.3324/haema- tol.11419] [PMID]
[13] Fucharoen S, Tunthanavatana C, Sonakul D, Wasi P. Intracranial extramedullary hematopoiesis in beta-thalassemia/hemoglobin E disease. American Journal of Hematology. 1981; 10(1):75-8. [DOI:10.1002/ajh.2830100111] [PMID]
[14] Teufel M, Bares R, Ernemann U, Horger M. [Extra-medullary hemat- opoiesis: Imaging findings (German)]. RöFo. 2008; 180(9):775-80. [DOI:10.1055/s-0028-1085533] [PMID]
[15] Lee CM, Salzman KL, Blumenthal DT, Gaffney DK. Intracranial extramedullary hematopoiesis: Brief review of response to radia- tion therapy. American Journal of Hematology. 2005; 78(2):151-2. [DOI:10.1002/ajh.20177] [PMID]
[16] Reddy R. Intracranial extramedullary hematopoiesis in primary myelofibrosis. Neurology India. 2017; 65(4):913-4. [DOI:10.4103/ neuroindia.NI_1151_16] [PMID]
[17] Ruiz-Cordero R, Jorgensen JL, Krishnamurthy S, Landon G. A common complication of myelofibrosis presenting as a rare find- ing in cerebrospinal fluid cytology. Diagnostic Cytopathology. 2017; 45(11):1039-41. [DOI:10.1002/dc.23768] [PMID]
[18] Mehta S, Shapey J, Pohl U, Vindlacheruvu R. Intracranial ex- tramedullary haematopoiesis: A case report. British Journal of Neu-rosurgery. 2015; 29(5):734-6. [DOI:10.3109/02688697.2015.10237 81] [PMID]
[19] Palma JA, Dominguez PD, Riverol M. Intracranial extramedullary hematopoiesis associated with multiple myeloma. Neurology. 2013; 80(17):1620. [DOI:10.1212/WNL.0b013e31828f1899] [PMID]
[20] Eskazan AE, Ar MC, Baslar Z. Intracranial extramedullary hemat- opoiesis in patients with thalassemia: A case report and review of the literature. Transfusion. 2012; 52(8):1715-20. [DOI:10.1111/ j.1537-2995.2011.03499.x] [PMID]
[21] Jiang WX, Zheng SQ, Wang PJ. 37-year-old woman with mul- tiple intracranial masses. Brain Pathology. 2011; 21(5):607-10. [DOI:10.1111/j.1750-3639.2011.00521.x] [PMID] [PMCID]
[22] Eskazan AE, Ar MC, Baslar Z. Intracranial mass in a patient with thalassaemia major. British Journal of Haematology. 2011; 152(2):126. [DOI:10.1111/j.1365-2141.2010.08493.x] [PMID]
[23] Zona G, Spena G, Sbaffi PF, Spaziante R. A meningioma with islets of extramedullary myeloid metaplasia: Case report. Neurosurgery. 2007; 61(2):E418-9. [DOI:10.1227/01.NEU.0000255520.04462.5A] [PMID]
[24] Shishime M, Suzumiya J, Takamatsu Y, Tamura K. [Intracranial extramedullary hematopoiesis following bilateral chronic subdural hematoma in a patient with idiopathic myelofibrosis (Japanese)]. The Japanese Journal of Clinical Hematology. 2005; 46(10):1160-2. [PMID]
[25] Ozdogu H, Boga C. Meningeal extramedullar haematopoiesis. British Journal of Haematology. 2004; 127(2):125. [DOI:10.1111/ j.1365-2141.2004.05136.x] [PMID]
[26] Ayyildiz O, Isikdogan A, Celik M, Muftuoglu E. Intracranial me- ningeal extramedullary hematopoiesis inducing serious headache in a patient with idiopathic myelofibrosis. Journal of Pediatric He- matology/Oncology. 2004; 26(1):28-9. [DOI:10.1097/00043426- 200401000-00009] [PMID]
[27] Beckner ME, Lee JYK, Schochet Jr SS, Chu CT. Intracranial ex- tramedullary hematopoiesis associated with pilocytic astrocy- toma: A case report. Acta Neuropathologica. 2003; 106(6):584-7. [DOI:10.1007/s00401-003-0767-1] [PMID]
[28] Ohtsubo M, Hayashi K, Fukushima T, Chiyoda S, Takahara O. Case report: Intracranial extramedullary haematopoiesis in postpoly- cythemic myelofibrosis. The British Journal of Radiology. 1994; 67(795):299-302. [DOI:10.1259/0007-1285-67-795-299] [PMID]
[29] Urman M, O'Sullivan RA, Nugent RA, Lentle BC. Intracranial ex- tramedullary hematopoiesis CT and bone marrow scan findings. Clin- ical Nuclear Medicine. 1991; 16(6):431-4. [DOI:10.1097/00003072- 199106000-00011] [PMID]
[30] Landolfi R, Colosimo Jr C, de Candia E, Castellana MA, de Cris- tofaro R, Trodella L, et al. Meningeal hematopoiesis causing exophthalmus and hemiparesis in myelofibrosis: Effect of radi- otherapy: A case report. 1988; 62(11):2346-9. [DOI:10.1002/1097- 0142(19881201)62:11<2346::aid-cncr2820621115>3.0.co;2-e] [PMID]
[31] Fucharoen S, Suthipongchai S, Poungvarin N, Ladpli S, Sonakul D, Wasi P. Intracranial extramedullary hematopoiesis induc- ing epilepsy in a patient with ß-thalassemia-hemoglobin E. Ar- chives of Internal Medicine. 1985; 145(4):739-42. [DOI:10.1001/ archinte.1985.00360040173037] [PMID]
[32] Lund RE, Aldridge NH. Computed tomography of intracranial ex- tramedullary hematopoiesis. Journal of Computer Assisted Tomog-raphy. 1984; 8(4):788-90. [DOI:10.1097/00004728-198408000- 00042] [PMID]
[33] Gregorios JB, Bay JW, Dudley Jr AW. Extramedullary hematopoie- sis in a malignant meningioma. Neurosurgery. 1983; 13(4):447-51. [DOI:10.1227/00006123-198310000-00020] [PMID]
[34] Kandel RA, Pritzker KP, Gordon AS, Bilbao JM. Extramedullary hematopoiesis simulating parasagittal meningioma. Canadian Journal of Neurological Sciences. 1982; 9(1):49-51. [DOI:10.1017/ S0317167100043638] [PMID]
[2] Koch BL, Bisset GS, Bisset RR, Zimmer MB. Intracranial extramedul- lary hematopoiesis: MR findings with pathologic correlation. Ameri- can Journal of Roentgenology. 1994; 162(6):1419-20. [DOI:10.2214/ ajr.162.6.8192010] [PMID]
[3] Haidar S, Ortiz-Neira C, Shroff M, Gilday D, Blaser S. Intracranial involvement in extramedullary hematopoiesis: Case report and review of the literature. Pediatric Radiology. 2005; 35(6):630-4. [DOI:10.1007/s00247-004-1361-y] [PMID]
[4] Kaya H. Cerci SS. Tc-99m nanocolloid scintigraphic imaging of in- tracranial meningeal extramedullary hematopoiesis in a patient with idiopathic myelofibrosis. Annals of Nuclear Medicine. 2006; 20(8):565-8. [DOI:10.1007/BF03026823] [PMID]
[5] Singer A, Quencer R. Intracranial extramedullary hematopoiesis: A rare cause of headaches. Journal of Neuroimaging. 2014; 24(5):524-7. [DOI:10.1111/jon.12029] [PMID]
[6] Kim CH. Homeostatic and pathogenic extramedullary hematopoie- sis. Journal of Blood Medicine. 2010; 1:13-9. [DOI:10.2147/JBM. S7224] [PMID] [PMCID]
[7] 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. Amer- ican Journal of Neuroradiology. 1997; 18(2):363-6. [PMID] [PMCID]
[8] Karki B, Xu YK, Tamrakar K, Wu YK. Intracranial extramedullary he- matopoiesis in beta-thalassemia. Korean Journal of Radiology. 2012; 13(2):240-3. [DOI:10.3348/kjr.2012.13.2.240] [PMID] [PMCID]
[9] Wang A, Carberry N, Solli E, Gillick J, Islam H, Hillard V. Spinal cord compression secondary to extramedullary hematopoiesis: Case re- port and review of the literature. Case Reports in Oncolog. 2016; 9(2):290-7. [DOI:10.1159/000446473] [PMID] [PMCID]
[10] Merchant R, Choudhari AJ, Verma M, Patkar DP, Doctor P. Intrac- ranial hematopoiesis in beta thalassemia: A case series. The Indian Journal of Pediatrics. 2018; 85(8):679-81. [DOI:10.1007/s12098- 018-2618-z] [PMID]
[11] Tayari N, Ahrar MH, Jafarpishe MS. Case report of the extramedul- lary hematopoiesis presented as a hypervascular intracranial mass. Advanced Biomedical Research. 2013; 2:34. [DOI:10.4103/2277- 9175.109719] [PMID] [PMCID]
[12] Musolino A, Guazzi A, Lazzaretti M, Pezzuolo D, Calzetti C, Degli Antoni A, et al. Intracranial hematopoiesis in a patient with AIDS- related central nervous system lymphoma and severe pancytope- nia. Haematologica. 2007; 92(5):e59-61. [DOI:10.3324/haema- tol.11419] [PMID]
[13] Fucharoen S, Tunthanavatana C, Sonakul D, Wasi P. Intracranial extramedullary hematopoiesis in beta-thalassemia/hemoglobin E disease. American Journal of Hematology. 1981; 10(1):75-8. [DOI:10.1002/ajh.2830100111] [PMID]
[14] Teufel M, Bares R, Ernemann U, Horger M. [Extra-medullary hemat- opoiesis: Imaging findings (German)]. RöFo. 2008; 180(9):775-80. [DOI:10.1055/s-0028-1085533] [PMID]
[15] Lee CM, Salzman KL, Blumenthal DT, Gaffney DK. Intracranial extramedullary hematopoiesis: Brief review of response to radia- tion therapy. American Journal of Hematology. 2005; 78(2):151-2. [DOI:10.1002/ajh.20177] [PMID]
[16] Reddy R. Intracranial extramedullary hematopoiesis in primary myelofibrosis. Neurology India. 2017; 65(4):913-4. [DOI:10.4103/ neuroindia.NI_1151_16] [PMID]
[17] Ruiz-Cordero R, Jorgensen JL, Krishnamurthy S, Landon G. A common complication of myelofibrosis presenting as a rare find- ing in cerebrospinal fluid cytology. Diagnostic Cytopathology. 2017; 45(11):1039-41. [DOI:10.1002/dc.23768] [PMID]
[18] Mehta S, Shapey J, Pohl U, Vindlacheruvu R. Intracranial ex- tramedullary haematopoiesis: A case report. British Journal of Neu-rosurgery. 2015; 29(5):734-6. [DOI:10.3109/02688697.2015.10237 81] [PMID]
[19] Palma JA, Dominguez PD, Riverol M. Intracranial extramedullary hematopoiesis associated with multiple myeloma. Neurology. 2013; 80(17):1620. [DOI:10.1212/WNL.0b013e31828f1899] [PMID]
[20] Eskazan AE, Ar MC, Baslar Z. Intracranial extramedullary hemat- opoiesis in patients with thalassemia: A case report and review of the literature. Transfusion. 2012; 52(8):1715-20. [DOI:10.1111/ j.1537-2995.2011.03499.x] [PMID]
[21] Jiang WX, Zheng SQ, Wang PJ. 37-year-old woman with mul- tiple intracranial masses. Brain Pathology. 2011; 21(5):607-10. [DOI:10.1111/j.1750-3639.2011.00521.x] [PMID] [PMCID]
[22] Eskazan AE, Ar MC, Baslar Z. Intracranial mass in a patient with thalassaemia major. British Journal of Haematology. 2011; 152(2):126. [DOI:10.1111/j.1365-2141.2010.08493.x] [PMID]
[23] Zona G, Spena G, Sbaffi PF, Spaziante R. A meningioma with islets of extramedullary myeloid metaplasia: Case report. Neurosurgery. 2007; 61(2):E418-9. [DOI:10.1227/01.NEU.0000255520.04462.5A] [PMID]
[24] Shishime M, Suzumiya J, Takamatsu Y, Tamura K. [Intracranial extramedullary hematopoiesis following bilateral chronic subdural hematoma in a patient with idiopathic myelofibrosis (Japanese)]. The Japanese Journal of Clinical Hematology. 2005; 46(10):1160-2. [PMID]
[25] Ozdogu H, Boga C. Meningeal extramedullar haematopoiesis. British Journal of Haematology. 2004; 127(2):125. [DOI:10.1111/ j.1365-2141.2004.05136.x] [PMID]
[26] Ayyildiz O, Isikdogan A, Celik M, Muftuoglu E. Intracranial me- ningeal extramedullary hematopoiesis inducing serious headache in a patient with idiopathic myelofibrosis. Journal of Pediatric He- matology/Oncology. 2004; 26(1):28-9. [DOI:10.1097/00043426- 200401000-00009] [PMID]
[27] Beckner ME, Lee JYK, Schochet Jr SS, Chu CT. Intracranial ex- tramedullary hematopoiesis associated with pilocytic astrocy- toma: A case report. Acta Neuropathologica. 2003; 106(6):584-7. [DOI:10.1007/s00401-003-0767-1] [PMID]
[28] Ohtsubo M, Hayashi K, Fukushima T, Chiyoda S, Takahara O. Case report: Intracranial extramedullary haematopoiesis in postpoly- cythemic myelofibrosis. The British Journal of Radiology. 1994; 67(795):299-302. [DOI:10.1259/0007-1285-67-795-299] [PMID]
[29] Urman M, O'Sullivan RA, Nugent RA, Lentle BC. Intracranial ex- tramedullary hematopoiesis CT and bone marrow scan findings. Clin- ical Nuclear Medicine. 1991; 16(6):431-4. [DOI:10.1097/00003072- 199106000-00011] [PMID]
[30] Landolfi R, Colosimo Jr C, de Candia E, Castellana MA, de Cris- tofaro R, Trodella L, et al. Meningeal hematopoiesis causing exophthalmus and hemiparesis in myelofibrosis: Effect of radi- otherapy: A case report. 1988; 62(11):2346-9. [DOI:10.1002/1097- 0142(19881201)62:11<2346::aid-cncr2820621115>3.0.co;2-e] [PMID]
[31] Fucharoen S, Suthipongchai S, Poungvarin N, Ladpli S, Sonakul D, Wasi P. Intracranial extramedullary hematopoiesis induc- ing epilepsy in a patient with ß-thalassemia-hemoglobin E. Ar- chives of Internal Medicine. 1985; 145(4):739-42. [DOI:10.1001/ archinte.1985.00360040173037] [PMID]
[32] Lund RE, Aldridge NH. Computed tomography of intracranial ex- tramedullary hematopoiesis. Journal of Computer Assisted Tomog-raphy. 1984; 8(4):788-90. [DOI:10.1097/00004728-198408000- 00042] [PMID]
[33] Gregorios JB, Bay JW, Dudley Jr AW. Extramedullary hematopoie- sis in a malignant meningioma. Neurosurgery. 1983; 13(4):447-51. [DOI:10.1227/00006123-198310000-00020] [PMID]
[34] Kandel RA, Pritzker KP, Gordon AS, Bilbao JM. Extramedullary hematopoiesis simulating parasagittal meningioma. Canadian Journal of Neurological Sciences. 1982; 9(1):49-51. [DOI:10.1017/ S0317167100043638] [PMID]
| Files | ||
| Issue | Vol 6 No 4 (2021): July-August | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/crcp.v6i4.7851 | |
| Keywords | ||
| Hematopoiesis Extramedullary cell differentiation Thalassemia | ||
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How to Cite
1.
Babaee M, Mohseni I, Mohammadi-Vajari M, Tefagh G, Rakhshankhah N, Motevalli M. Extramedullary Hematopoiesis Presenting as Progressive Headache: A Case Report and Review of Literature. CRCP. 2021;6(4):124-136.
