[Intravascular malignant lymphomatosis].
Based on our personal experience of two cases of intravascular malignant lymphomatosis (IML) and a review of 128 cases in the literature, the possibilities for clinical diagnosis and successful treatment of this condition are discussed. IML usually presents with hemiparesis or progressive dementia and then with disturbances of consciousness. Paraparesis and dysuria due to spinal cord involvement are not rare. Since increased levels of blood sedimentation rate, CRP, serum LDH, and CSF protein are often observed in the patients, a diagnosis of IML must be included among the list of differential diagnoses when these findings are encountered in a patient presenting with clinical features similar to those of cerebrovascular diseases or multiinfarct dementia. The diagnosis must be confirmed histologically, so that an appropriate treatment can be initiated early in the course of the disease. Skin biopsy is indicated when skin eruption is present. Autopsy records of patients have revealed involvement of intravascular lymphoma to the kidney, adrenals, lungs and liver in more than 80% of cases. It is suggested therefore that biopsies are performed for these organs. Muscle biopsy can also be useful for making a diagnosis. Since autopsy studies have revealed swelling of the adrenals in some cases, including ours, CT or MRI images of the adrenals might be of importance. Regarding treatment, chemotherapy must be indicated. However, this has so far shown either no or only a temporary efficacy, partly because of the delay in reaching a diagnosis. A cure for IML could be expected through early initiation of combined treatment with various chemotherapeutic agents in the near future.
Koto
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Department of Neurology, School of Medicine, Keio University.
D000368:Aged; D000369:Aged, 80 and over; D001808:Blood Vessels / Q000473:pathology; D005260:Female; D006801:Humans; D007238:Infarction / Q000209:etiology / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D014652:Vascular Diseases / Q000150:complications / Q000175:diagnosis / Q000473:pathology*