Abstract
Background: Intravascular lymphoma is a rare and aggressive form of non-Hodgkin lymphoma defined pathologically by neoplastic proliferation of lymphoid cells within the lumens of small to medium sized blood vessels, with little or no parenchymal involvement. Most intravascular lymphomas are of B-cell origin while a minority are T-cell. Neoplastic cells localize within vascular lumina but rarely infiltrate other areas associated with lymphoma such as lymph nodes, bone marrow, or peripheral blood. The mechanism for the intravascular localization of these cells remains unexplained. Intravascular lymphoma has an extremely poor prognosis; usually patients exhibit nonspecific clinical presentations that make diagnosis difficult. We report a series of three patients with intravascular lymphoma at our institution with a review of the published literature and discuss its presentation and clinical implications.
Cases:
A 62 year old male presented with lower extremity weakness and numbness associated with bowel and bladder incontinence. MRI of his brain and thoracic spine revealed advanced chronic ischemic changes with areas of acute infarct, and T4-T6 transverse myelitis, respectively. He developed fevers, had one positive blood culture, and clinically demonstrated a progressive neurologic deterioration. Autopsy revealed intravascular large B-cell lymphoma cells involving small vessels within the liver, spleen, kidneys, and most extensively within the brain & spinal cord. Vascular occlusion by lymphoma cells in the brain & spinal cord led to multiple infarcts and thus a rapid neurologic decline. A 76 year old female presented with a two month history of fever, malaise, weight loss, and generalized weakness. She was found to have ascites, anasarca, and hypoalbuminemia. All cultures and diagnostic tests were unrevealing. She demonstrated multi-organ system failure and deteriorated, expiring on hospital day eleven. Post mortem exam revealed intravascular large B-cell lymphoma in small vessels within the colon, liver, epicardium, lungs, and hepatic sinusoids. Small vessels in soft tissue adjacent to the thyroid and adrenal glands also contained malignant cells. A 58 year old female presented with a two year history of recurring erythematous, tender, thickened areas of the lower extremities associated with fevers and malaise. Skin biopsy of an indurated thigh lesion revealed intravascular lymphoma. She was treated with six cycles of CHOP chemotherapy and achieved complete clinical remission with no evidence of recurrence over many years of close surveillance.
Methods: A PubMed search, English language medical literature, using key words “intravascular lymphoma” and “angiotrophic lymphoma.”
Conclusion: Intravascular lymphoma is a rare type of lymphoma with a poor prognosis. It has an aggressive clinical course with proliferation of neoplastic cells within blood vessels of organs, resulting in luminal thrombosis and thus multi organ system manifestations, although there is a higher incidence of cutaneous and central nervous system abnormalities. The medical literature indicates that few cases are diagnosed antemortem because often presenting signs are unlike those of lymphoma. There is favorable response to chemotherapy provided the diagnosis is considered and made. The diagnosis of intravascular lymphoma must be considered in the patient who presents with fever of unknown origin associated with neurologic signs and /or skin changes. Progression of the disease usually involves multi organ system manifestations with only rare involvement of lymph nodes and bone marrow. Diagnosis is confirmed by biopsy.
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