Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a typical case of intravascular lymphomatosis, a rarely diagnosed, generalised intravascular lymphoma usually of the B-cell type. In most cases there is a lack of clear haematological findings but in more than 50% intravascular lymphomatosis presents with symptoms of the central nervous system. Every rapidly progressive neurological deficit, especially the association of a subacute dementia with a spinal syndrome may suggest IVL-NS. However, careful examination may detect minor features for a systemic process in 25-80% i.e. B-symptoms elevation of ESR and LDH. Neurological imaging demonstrates multifocal lesions in the CNS with affinity to the deep white matter consistent with a microvascular or demyelinating disease. Angiographically IVL-NS may mimic cerebral vasculitis. CSF findings are nonspecific. Because diagnosis can only be made histologically, a cerebral biopsy should be undertaken in suspected cases. Usually the course of the disease is fatal. Therapy involving steroids, combination polychemotherapy or radiation met with only minor success.
 ...
PMID:[Intravascular lymphomatosis of the nervous system--case report and review of the literature]. 876 97

The neuropathological findings of IVL are characterized by multiple infarctions involving the cerebral cortex and white matter, and infarctions, that do not affect the areas of major arterial supply. Histologically, the disorder is characterized by cerebral necrosis due to intravascular proliferation of tumor cells, with pronounced angiitis or thrombosis. IVL is also associated with frequent spinal cord involvement. At the time of recurrence increasing number of patients with IVL present with findings indistinguishable from those of primary malignant lymphoma of the brain, because advances in the treatment of IVL have increased the follow-up period of these patients. Intravascular proliferation of tumor cells in various organs is found in patients with IVL. The tumor cells also show extravascular proliferation and form tumor masses in the liver or adrenal gland. Neurological involvement of malignant lymphoma other than IVL includes primary malignant lymphoma of the brain, lymphomatous meningitis, neurolymphomatosis, and spinal cord compression from epidural metastases. We herein describe the differences between these disorders and IVL.
 ...
PMID:[Pathology of intravascular lymphoma]. 2151 25

IVL is characterized by a propensity for intravascular tumor cell proliferation. Premortem diagnosis of IVL is difficult because of its nonspecific clinical, laboratory, and imaging manifestations. This study examined cerebral MR imaging patterns of IVL and their changes with and without chemotherapy. Nine of 11 patients studied presented with abnormal findings. We define 5 patterns of abnormal MR imaging findings: 1) infarctlike lesions, 2) nonspecific white matter lesions, 3) meningeal enhancement, 4) masslike lesions, and 5) hyperintense lesions in the pons on T2WI. Seven patients presented with only 1 pattern, while 2 patients presented with multiple patterns. Lesions in 7 treated patients responded to chemotherapy. Pathologic specimens revealed intravascular tumor cell infiltration with associated infarctions, necrosis, congestion, demyelination, vasculitis, and tumor cell extravasation. We conclude that MR imaging patterns can be possible manifestations of intravascular-dominant infiltration by tumor cells with associated occlusion or inflammation, depending on the level of affected vessels.
 ...
PMID:Characteristics of intravascular large B-cell lymphoma on cerebral MR imaging. 2217 63