8th Baltic Congress of Radiology

Neuroradiology

poster abstract

Intravascular lymphoma (IVL) is a rare, often fatal disease characterized by the intraluminal proliferation of lymphomatous cells within the blood vessels. [1] Intravascular large B-cell lymphoma (IVLBCL) accounts for 1% of B- cell lymphomas. From the Surveillance, Epidemiology, and End Results (SEER) data in the USA, the incidence of IVLBCL is estimated to be 0.095 per 1000 000 per year. The increase in IVLBCL incidence is inferred due to a more precise diagnosis and improved awareness of the disease. [4] The median age at the time of the presentation of the disease is described as 70 years (ranging 34-90 years). Men and women are equally affected. [5] IVLBCL is frequently found with multiple organs, but the skin and central nervous system (CNS) are the most commonly affected ones. [3] Abnormal findings on brain magnetic resonance imaging (MRI) are found approximately 90% of patients with IVLBCL. [7] Overall, the prognosis is poor with a high mortality rate, average survival times in some cases between 5 and 7 months. However, recent data suggest that 50% 5-year survival can be achieved if treatment is initiated in the early stages of the disease. [2] The accurate diagnosis of IVLBCL limited to the CNS is challenging and depends primarily on histopathological examination due to the lack of specific clinical manifestations, laboratory markers, and radiological features. Appropriate treatment can improve outcomes, making a timely and correct diagnosis crucial for patients with IVLBCL. [6] The main differential diagnosis for intravascular large B-cell lymphoma includes intravascular natural killer cell (NK) lymphomas, intravascular T-cell lymphomas, and intralymphatic ALK-negative anaplastic large cell lymphomas (ALCL). [2] Also IVLBCL should be differentiated from central pontine myelinolysis, posterior reversible encephalopathy syndrome,inflammatory diseases, such as infections, autoimmune vasculitis, IgG4-related diseases and age-related changes. [8]

We report a large B-cell lymphoma patient with rare case of CNS involvement. The patient underwent laboratory and radiological examinations. High-resolution, contrast-enhanced T1-weighted (T1WI), T2-weighted (T2WI) and FLAIR-weighted head magnetic resonance (MRI) together with magnetic resonance diffusion weighted imaging (DWI) and perfusion imaging (PWI) studies was used to confirm the diagnosis.

A 63-year-old female presented with complaints about the progressive cognitive deficit (she could not remember her door code, could not count, and had speech difficulties). It was known that the patient had chronic B-cell lymphocytosis, II stage (2017), 2 months previously splanchnic venous thrombosis, idiopathic focal epilepsy. A neurological examination revealed acalculia, agraphia, senso-motor aphasia, apraxia. Blood biochemical analysis without any significant change. In full blood count, Gumprecht shadows could be seen. Additionally, an analysis on anti-HIV 1/2, anti-HCV, HbsAg, RPR, and TPHA syphilis tests were negative. Lumbar puncture showed no significant changes - cell count 0, protein 0.57 g/L. Initial head computed tomography (CT) showed a small hypodense zone in the left parietal lobe. Head magnetic resonance imaging (MRI) was indicated for further investigation. MRI of the brain showed pathological signal areas on the left parietal lobe with hypointense signal on contrast-enhanced T1-weighted (T1WI), hyperintense signal on contrast-enhanced T2-weighted (T2WI) and FLAIR-weighted MRI with restricted diffusion and slightly lower ADC value. Magnetic resonance perfusion imaging studies did not show increase in cerebral blood flow (CBF) or in cerebral blood volume (CBV).

The disease progresses mainly with nonspecific symptoms such as fever, respiratory symptoms, and general fatigue which makes timely diagnosis very difficult. [4] The main clinical sign of IVLBCL is a lack of lymphadenopathy. [6] More than 60% of patients with IVLBCL are reported to develop neurological symptoms during the clinical course of the disease. [6] In this case, the patient's diagnosis was confirmed based on typical radiological features. Tipical MRI findings for IVLBCL are - T1WI: multifocal hypointense lesions; T2WI: majority show hyperintensities in deep white matter, mild surrounding edema is typical; FLAIR: homogeneously iso-/hypointense, may be hyperintense; DWI: often show restricted diffusion with low ADC values; PWI: low CBV and CBF ratios; T1WI C+: variable enhancement, meningeal and/or dural enhancement. [9]

We present the case of a patient with large B-cell lymphoma with rare case of CNS involvement. A 63-year-old female presented with complaints about the progressive cognitive deficit. Biochemical analysis of the blood and lumbar puncture did not show significant changes. Head MRI revealed pathological lesion in the left parietal lobe with restricted diffusion and slightly lower ADC value, perfusion studies did not show an increase in CBF or CBV.

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