Intravascular lymphomatosis was originally described in 1959 by Pfleger and Tappeiner. They reported a young woman with multiple skin nodules, which microscopically demonstrated distended vessels filled with atypical cells, associated with occlusion of the vessels, and suggested that the lesions might be endothelial in origin. The disorder is known by a variety of other names, including intravascular malignant lymphomatosis; malignant angioendotheliomatosis; proliferating angioendotheliomatosis; angiotropic large-cell lymphoma; malignant angioendotheliomatosis fulminans; and neoplastic angioendotheliomatosis.
Intravascular lymphomatosis is a rare angiotrophic large-cell lymphoma that produces vascular occlusion of arterioles, capillaries, and venules subsequent to the proliferation of neoplastic lymphoid cells. Antigenic phenotyping shows that these lymphomas are primarily B-cell and less commonly T-cell lymphomas. The central nervous system and skin are the most commonly affected organs.
Patients usually present with progressive encephalopathy and focal neurologic deficits associated with skin findings, such as petechia or purpura. Other involved organs include the adrenal glands, lungs, heart, spleen, liver, pancreas, genital tract, and kidneys. Bone marrow, blood, CSF, and lymph nodes are typically spared.
The typical findings in patients with intravascular lymphomatosis include the following:
Frequent laboratory findings:
Pathologically, affected vessels are distended and occluded with large, mononuclear, predominantly B cells (CD 20, CD 19, CD 22, and CD 79 alpha-positive). The nuclear contours are irregular in these cells, with 2 or more nuclei per cell and moderate amounts of basophilic cytoplasm resulting in high nuclear:cytoplasm ratios, with frequent mitotic figures.
Most cases of intravascular lymphomatosis are progressive, but spontaneous remissions have been reported. Typically, however, the mean survival is 6 months from the onset of symptoms. The administration of steroids can cause brief remissions.
In a review of 77 patients with symptoms of intravascular lymphomatosis, only 22 (29%) had received a premortem diagnosis, primarily because of the nonspecific nature of clinical manifestations and the rarity of the condition. The majority of patients experience progressive multifocal events suggesting ischemia in a small artery distribution.
CHOP chemotherapy (the combination of cylcophosphamide, adriamycin, vincristine, and prednisone) leads to remission in approximately 55% of patients, but relapses are common. The success rate is significantly affected by the patient's pretreatment clinical status. Two reports describe successful outcomes following autologous peripheral blood stem cell transplantation after a post-CHOP relapse.
Unfortunately, the patient in this case continued to decline after his biopsy. Given his poor prognosis and severe debilitation, his family elected to transfer his care to hospice where he died shortly thereafter.
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