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|Year : 2017 | Volume
| Issue : 4 | Page : 913-914
Intracranial extramedullary hematopoiesis in primary myelofibrosis
Department of Radiology, St. John's Medical College, Bengaluru, Karnataka, India
|Date of Web Publication||5-Jul-2017|
Department of Radiology, St. John's Medical College, Bengaluru - 560034, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Reddy R. Intracranial extramedullary hematopoiesis in primary myelofibrosis. Neurol India 2017;65:913-4
A 40-year old gentleman was suffering from myelofibrosis for 2 years with liver and spleen involvement. He had undergone splenectomy 8 months prior, and presented with complaints of headache and diplopia for 2 weeks. Due to the severity of this condition, he was referred for magnetic resonance imaging (MRI) of the brain. Cranial MRI showed T1 hypointense [Figure 1]a parafalcine and paratentorial lobulated soft tissue lesions, which showed gradient blooming on T2 gradient recalled echo (GRE) images [Figure 1]b and homogenous enhancement after intravenous injection of gadopentetate dimeglumine [Figure 1]c. These soft tissue masses showed no perilesional edema. The calvarium was thickened with diploic space widening and showed multiple subcentimetric well-circumscribed enhancing lesions [Figure 1]d. These calvarial lesions were noted to be lytic on computed tomography (CT) [Figure 1]e. These findings were consistent with features of extramedullary hematopoiesis (EMH). In our case, the peripheral smear showed classic findings of leukoerythroblastosis and giant platelets. Our differential diagnosis was EMH, subdural hematoma, meningioma, and meningeal metastasis, in order of priority. The MRI features with laboratory findings could be extrapolated to the clinical picture, thus reinforcing the diagnosis of EMH of the falx cerebri and tentorium. The hypointensity on T1 and T2-weighted images on MRI was attributed to magnetic susceptibility effect caused by hemosiderin. Both imaging features and clinical findings led to this diagnosis [Table 1].
|Figure 1: (a) Axial T1-weighted magnetic resonance image of the brain showing a hypointense, parafalcine lobulated soft tissue lesion. (b) Axial GRE T2 magnetic resonance image of the brain showing gradient blooming of the parafalcine soft tissue lesion secondary to hemosiderin deposition. (c) Axial T1 contrast magnetic resonance image of the brain showing homogenous enhancement of the parafalcine soft tissue lesion after intravenous injection of gadopentetate dimeglumine. (d) Axial T1 contrast magnetic resonance image of the brain showing thickening of the calvarium with diploic space widening and multiple subcentimetric well-circumscribed enhancing lesions. (e) Axial computed tomography image of the calvarium in bone window showing the lytic nature of subcentimetric enhancing lesions demonstrated in (d)|
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In EMH, a compensatory response by proliferation of the pleuripotent mesenchymal stem cells outside the bone marrow is attempted by the body to meet the body's demand in the presence of anemia. MRI is the diagnostic investigation of choice. Intracranial EMH is characterized by multiple iso- or hyperintense extra-axial masses appended to the meninges, with homogeneous enhancement after contrast administration. EMH in the intracranial or intraspinal epidural space can lead to serious neurogenic complications (increased intracranial pressure, hemiplegia, altered levels of consciousness or visual disturbances, including subdural hemorrhage, delirium, increased intracranial pressure, papilledema, coma, motor and sensory impairment, and limb paralysis caused by direct mass effect upon adjacent structures). It has also been reported in association with an intracranial hemangioblastoma and a meningioma.
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