Intravascular Large B-Cell Lymphoma (IVLBCL) Presenting with CNS Involvement in Patient with Chronic Lymphocytic Leukemia

Abstract

Background: Intravascular lymphoma (IVL) is a rare, often fatal disease characterized by intraluminal proliferation of lymphoid cells within blood vessels. Intravascular Large B-Cell Lymphoma (IVLBCL) is frequently found in different organs, but the skin and central nervous system (CNS) are the most affected ones. Routine radiological examination and additional diagnostic tools, such as high-resolution, three-dimensional (3D) FLAIR sequence, T2-weighted (T2W) and T1-weighted (T1W) contrast-enhanced magnetic resonance imaging (MRI), MRI diffusion and perfusion imaging studies are useful to confirm the diagnosis in cases where it is not easy to perform histopathological examinations [1]. 1.2. Case Report: We present a clinical case of a 63-year-old female patient who presented with progressive cognitive deficit as mild motor aphasia, acalculia, agraphia, dyslexia and mild ideomotor apraxia without any signs of other organ involvement. It was known that the patient had 5-year anamnesis of chronic lymphocytic leukemia, stage II and splanchnic venous thrombosis 2 months ago. Blood biochemical analysis without any significant change, except leukocytosis in full blood count (leukocytes 15,2 x 10*9/L, where neutrophils were in normal range, but lymphocytes were increased). Head magnetic resonance imaging (MRI) showed pathological signal areas on the left parietal lobe with hypointense signal on contrast-enhanced T1-weighted, hyperintense signal on contrast-enhanced T2-weighted and FLAIR-weighted MRI images with restricted diffusion and slightly lower ADC value. Magnetic resonance perfusion imaging studies did not show an increase in cerebral blood flow or in cerebral blood volume. In this case, the patient’s diagnosis was made based on typical radiological features. 1.3. Conclusions: Intravascular large B-cell lymphoma is a rare subtype of extra nodal large B-cell lymphoma with an aggressive clinical course. The diagnosis is challenging, as it presents without any obvious tumour mass or lymphadenopathy. [2] As the clinical presentation is variable without specific characteristics, mostly biopsy or even autopsy confirms the definite diagnosis. But in some cases, the diagnosis can be made by typical radiological findings in the restricted availability of brain biopsy.