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An intracranial extramedullary hematopoiesis in a 34-year-old man with beta thalassemia: a case report.

Tabesh H, Shekarchizadeh A, Mahzouni P, Mokhtari M, Abrishamkar S, Abbasi Fard S - J Med Case Rep (2011)

Bottom Line: Intracranial deposits of extramedullary hematopoiesis are an extremely rare compensatory process in intermediate and severe thalassemia.We present an unusual case of an intracranial extramedullary hematopoiesis with a choroid plexus origin in a 34-year-old Caucasian man with beta thalassemia intermedia, who presented with the complaints of chronic headache and rapid progressive visual loss.An intracranial extramedullary hematopoiesis, although extremely rare, should be considered as a potential ancillary diagnosis in any thalassemic patient and therefore appropriate studies should be performed to investigate the probable intracranial ectopic marrow before any surgical intervention.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Neurosurgery, Al-Zahra Hospital, Isfahan University Of Medical Sciences, Isfahan, Iran. Dr_abbasi_s@yahoo.com.

ABSTRACT

Introduction: Extramedullary hematopoiesis occurs in approximately 15% of cases of thalassemia. Intracranial deposits of extramedullary hematopoiesis are an extremely rare compensatory process in intermediate and severe thalassemia.

Case presentation: We present an unusual case of an intracranial extramedullary hematopoiesis with a choroid plexus origin in a 34-year-old Caucasian man with beta thalassemia intermedia, who presented with the complaints of chronic headache and rapid progressive visual loss.

Conclusion: An intracranial extramedullary hematopoiesis, although extremely rare, should be considered as a potential ancillary diagnosis in any thalassemic patient and therefore appropriate studies should be performed to investigate the probable intracranial ectopic marrow before any surgical intervention.

No MeSH data available.


Related in: MedlinePlus

Axial T1-weighted MRI image with IV contrast demonstrating a large homogenously enhanced lesion in the left trigone area.
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Figure 3: Axial T1-weighted MRI image with IV contrast demonstrating a large homogenously enhanced lesion in the left trigone area.

Mentions: In a skull X-ray, expansion of the diploic space with a so-called hair-on-end appearance inline with EMH was seen. Computed tomography (CT) study of his brain disclosed a large hyperdense lobulated lesion in the vicinity of the left occipital horn with significant surrounding edema which enhanced after intravenous contrast injection. Magnetic resonance imaging (MRI) of his brain revealed a hyperintense lesion in T1-weighted images which was signal void in T2-weighted imaging, with significant peripheral edema (Figures 1, 2 and 3). There was a left-to-right shift of the midline and thickening of the calvarium in the sagittal plane.


An intracranial extramedullary hematopoiesis in a 34-year-old man with beta thalassemia: a case report.

Tabesh H, Shekarchizadeh A, Mahzouni P, Mokhtari M, Abrishamkar S, Abbasi Fard S - J Med Case Rep (2011)

Axial T1-weighted MRI image with IV contrast demonstrating a large homogenously enhanced lesion in the left trigone area.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3285055&req=5

Figure 3: Axial T1-weighted MRI image with IV contrast demonstrating a large homogenously enhanced lesion in the left trigone area.
Mentions: In a skull X-ray, expansion of the diploic space with a so-called hair-on-end appearance inline with EMH was seen. Computed tomography (CT) study of his brain disclosed a large hyperdense lobulated lesion in the vicinity of the left occipital horn with significant surrounding edema which enhanced after intravenous contrast injection. Magnetic resonance imaging (MRI) of his brain revealed a hyperintense lesion in T1-weighted images which was signal void in T2-weighted imaging, with significant peripheral edema (Figures 1, 2 and 3). There was a left-to-right shift of the midline and thickening of the calvarium in the sagittal plane.

Bottom Line: Intracranial deposits of extramedullary hematopoiesis are an extremely rare compensatory process in intermediate and severe thalassemia.We present an unusual case of an intracranial extramedullary hematopoiesis with a choroid plexus origin in a 34-year-old Caucasian man with beta thalassemia intermedia, who presented with the complaints of chronic headache and rapid progressive visual loss.An intracranial extramedullary hematopoiesis, although extremely rare, should be considered as a potential ancillary diagnosis in any thalassemic patient and therefore appropriate studies should be performed to investigate the probable intracranial ectopic marrow before any surgical intervention.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Neurosurgery, Al-Zahra Hospital, Isfahan University Of Medical Sciences, Isfahan, Iran. Dr_abbasi_s@yahoo.com.

ABSTRACT

Introduction: Extramedullary hematopoiesis occurs in approximately 15% of cases of thalassemia. Intracranial deposits of extramedullary hematopoiesis are an extremely rare compensatory process in intermediate and severe thalassemia.

Case presentation: We present an unusual case of an intracranial extramedullary hematopoiesis with a choroid plexus origin in a 34-year-old Caucasian man with beta thalassemia intermedia, who presented with the complaints of chronic headache and rapid progressive visual loss.

Conclusion: An intracranial extramedullary hematopoiesis, although extremely rare, should be considered as a potential ancillary diagnosis in any thalassemic patient and therefore appropriate studies should be performed to investigate the probable intracranial ectopic marrow before any surgical intervention.

No MeSH data available.


Related in: MedlinePlus