Cerebral intraparenchymal extramedullary hematopoiesis in polycythemia vera
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Cerebral intraparenchymal extramedullary hematopoiesis in polycythemia vera Jasmina Boban1,3 · Peter Kalhs2 · Majda M. Thurnher1 Received: 23 November 2018 / Accepted: 23 July 2019 © The Author(s) 2019
Polycythemia vera (PV) is the most common myeloproliferative neoplasm, characterized by erythrocytosis, leukocytosis, and thrombocytosis and often associated with splenomegaly [1]. Neurological complications of PV are rare, but can include ischemic strokes, transient ischemic attacks (TIAs), and atypical TIAs [2]. Extramedullary hematopoiesis (EMH) is a compensatory physiological response of the organism to the failure of hematopoiesis at the bone marrow, which occurs commonly in patients with chronic hemolytic anemia or diseases with ineffective erythropoiesis [3]. Usually, extramedullary production of blood precursor cells occurs in the spleen, liver, or lymph nodes. However, cases with EMH in the adrenals, thymus, pleura, skin, gastrointestinal tract, and paranasal sinuses have been described [4]. Intracranial EMH is extremely rare, and only cases involving the dura mater have been described [3]. To the best of our knowledge, no cases of intracranial intraparenchymal EMH have been described. A 48-year-old man with known polycythemia vera for 6 years presented with acute tinnitus. Six months before this, he was diagnosed with disease progression due to splenomegaly, anemia, leukocytosis (100,000 cells/μl,), and thrombocytopenia. Magnetic resonance (MR) imaging of the brain was performed on a 1.5 T scanner (Philips Gyroscan Intera, NE), consisting of axial fluid attenuation inversion recovery (FLAIR), sagittal T1-weighted (T1WI), coronal T2-weighted * Majda M. Thurnher [email protected] 1
Department for Biomedical Imaging and Image‑guided Therapy, University Hospital Vienna, Medical University of Vienna, Waehringer Guertel 18‑20, 1090 Vienna, Austria
2
Department for Internal Medicine, University Hospital Vienna, Medical University of Vienna, Waehringer Guertel 18‑20, 1090 Vienna, Austria
3
Present Address: Faculty of Medicine, University of Novi Sad, Hajduk Veljkova 3, Novi Sad 21000, Serbia
(T2WI) MR images, diffusion-weighted imaging (DWI), and a post-contrast T1WI study in two planes. In the right temporal lobe, there was a T1WI isointense (Fig. 1b) and T2WI hypointense (Fig. 1c), intraparenchymal 2-cm-lesion, surrounded by a rim of T2W high-intensity perilesional edema. The lesion was adjacent to the right temporal horn. On DWI, the lesion showed a low signal. On post-contrast T1W images (Fig. 1d), there was a patchy, dot-like enhancement. There were no other brain lesions. Elective splenectomy was performed 2 days later, and histology revealed extramedullary hematopoiesis. On a follow-up brain MR scan, 6 weeks after the initial exam, the lesion showed a significant reduction in size, with central T1W- and T2W hyperintensity and a dark peripheral rim. No edema and no contrast enhancement were detected. Six weeks later, on the last follow-up MR scan, there was only a n
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