Learning objectives
Review the clinical manifestations of Intravascular Lymphoma (IVL).
Comment some laboratory procedures used in the diagnosis.
Describe the imaging findings in Computed Tomography (CT) and Magnetic Resonance Imaging (MR).
Background
IVL is a rare subtype of Lymphoma.
It is usually due to multifocal proliferation of tumor cells (large B-cells) in small vessels.
It has a predilection for central nervous system (CNS) and skin,
but it can affect kidneys,
bone marrow,
breast,
uterus,
testicles,
lungs,
larynx and adrenal glands.
It has been reported association with Epstein Barr virus also.
Usually,
it affects patients in their 6th to 7th decades of life,
with a male-female ratio of 2:1.
The survival is about 7 to 13 months with...
Findings and procedure details
Clinically,
IVL presents as fever of unknown origin or intermittent fevers.
Skin rash may be seen mainly in the abdomen and thighs in 50% of the cases.
When CNS involvement appears it manifests as subacute encephalopathy ranging from disorientation to focal signs such as hemiparesis and myelopathy,
dementia (most common form of presentation),
seizure,
multifocal cerebrovascular events or memory loss.
Among the procedures used for diagnosis are CSF analysis which may show mild leukocytosis or an increased amount of protein,
but cytologic results are negative....
Conclusion
IVL is a rare and fatal disease which consists in proliferation of aggressive lymphoma cells within the lumen of small blood vessels.
Althought ultimate diagnosis test is cerebral or skin biopsy,
as the disease progresses,
MR imaging might reveal some findings to help us make the diagnosis and improve the prognosis.
Even though imaging techniques findings are inespecific,
IVL should be suspected in patients with dementia,
multifocal lesions and enhacement without documented cerebrovascular disease or immunosuppression.
References
Yamamoto,
A and col; "Characteristic of Intravascular Large B-cell Lymphoma on Cerebral MR Imaging"; AJNR 33: 292-296.
February 2012.
Kawai,
Nobuyuki and col; "Insufficiency of Positron Emission Tomography and Magnetic Resonance Spectroscopy in the Diagnosis of Intravascular Lymphoma of the Central Nervous System"; Case Reports in Oncology 2012; 5:336-346.
July 2012.
Miyazaki,
Chihoko and col; "A Case of Intravascular Lymphoma with Increased Regional Cerebral Blod FLow in I-123 IMP Single-Photon Emission CT",
AJNR American Journal Neurorradiology 25:565-570.
April 2004.
Osborn; Intravascular Lymphoma,
Diagnostic Imaging,
Second...