Dominant neurologic symptomatology in intravascular large B-cell lymphoma

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare variant of extranodal large B-cell lymphoma and it is characterized by selective intravascular proliferation of malignant cells. Typical features of the disease include aggressive behavior, rapid and frequently fatal course. Clinical picture is non-specifi c and heterogeneous, depending on the affected organ. It is not uncommon that this unique type of lymphoma is diagnosed post mortem. Herein, we report two cases of IVLBCL with neurologic symptomatology. In our clinical study patient 1 was an 80-year-old male with mixed paraparesis of lower extremities and diffi culties with sphincter control. Patient 2 (56-year-old male) had vision malfunction, mental status changes and defect in phatic and motor functions. In both cases defi nite diagnosis was established by histological examination of necroptic material. We propose to include IVLBCL in differential diagnostic considerations in patients presenting with gradually impairing neurological status and spinal cord damage of unknown etiology (Fig. 2, Ref. 9). Text in PDF www.elis.sk.

Intravascular large B-cell lymphoma presenting as rapidly progressive dementia and stroke

“…[ 4 ] The most frequent laboratory test results of IVLBCL are anemia, high erythrocyte sedimentation rate, and elevated LDH. [ 14 ] Although various patterns of abnormal features on brain MRI in patients with IVLBCL have been reported, a recent study suggested the findings on brain MRI were categorized into 4 patterns: (1) non-specific white matter lesions, (2) infarct-like lesions, (2) hyperintense lesions in the pons, and (4) meningeal thickening and/or enhancement. [ 15 ] In contrast, brain MRA is not useful in the diagnosis of IVLBCL as affected cerebral vessels are too small to be directly shown on MRA.…”

Small Cell Variant of Intravascular Large B-Cell Lymphoma: Highlighting a Potentially Fatal and Easily Missed Diagnosis

“…Previous studies have shown that although any organ may be involved, neurological signs and symptoms are the most common presentation of the disease [ 3 – 8 ]. Neuropathic deficits are very heterogeneous, including mental status changes, transient ischemic attack, paraparesis of the extremities, and defect in motor functions [ 9 , 10 ]. In the current study, central nervous system, bone marrow, pericardium, liver, skin, small bowel, and fallopian tube and ovary were organs with biopsy-proven involvement by lymphoma.…”

Intravascular large B‐cell lymphoma affecting multiple cranial nerves: A histopathological study

“…Brain magnetic resonance imaging (MRI) reveals normal findings or nonspecific hyperintense lesions in the white matter, suggestive of demyelination or ischemic pathology by small vessel occlusion. [8][9][10] Laboratory tests are usually nonspecific. Malignant lymphoid cells are only occasionally found in the peripheral blood smear or in the cerebrospinal fluid, where an increased protein level and/or lymphocytosis are more common although unspecific.…”

“…Malignant lymphoid cells are only occasionally found in the peripheral blood smear or in the cerebrospinal fluid, where an increased protein level and/or lymphocytosis are more common although unspecific. 8,10 Intra vitam diagnosis is particularly difficult due to the rapid course of the disease, the lack of involvement of lymph nodes and bone marrow, and the unspecific clinical, laboratory and imaging findings. Biopsies are needed to reach a diagnosis, but their success depends on lymphoma localization.…”

“…Laboratory tests are usually nonspecific. Malignant lymphoid cells are only occasionally found in the peripheral blood smear or in the cerebrospinal fluid, where an increased protein level and/or lymphocytosis are more common although unspecific 8,10 …”