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© 2001 American Society for Clinical Oncology
Unusual Sites of Involvement by Hematologic MalignanciesCase 1. Intravascular Large B-Cell Lymphoma Presenting With CNS SymptomsBall Memorial Hospital, Muncie, IN A 59-year-old white woman presented with recurrent symptoms of numbness and weakness in the head and neck. A computed tomographic (CT) scan showed diffuse white matter disease in the cerebellum and posterior corpus callosum. Soon after, the patient was admitted to the hospital with increasing combative behavior, worsening mental status, ataxia, and left-sided paresis; a cerebrovascular accident was diagnosed. The patient was readmitted 2 months later for increasing confusion and ataxia. The CT findings remained unchanged. She continued to deteriorate, becoming more confused and unable to walk or swallow. Magnetic resonance imaging scans and CSF examinations were consistent with a diagnosis of multiple sclerosis. Prednisolone and sertraline hydrochloride were administered without benefit. She died 4 months after the initial presentation. Significant gross autopsy findings included coronary atherosclerosis, 500 mL of ascitic fluid in the peritoneal cavity, splenomegaly (530 g), and macular skin lesions on the upper and lower extremities and anterior chest. On examination of the brain, multiple poorly defined, granular, slightly soft white matter lesions in the cerebral hemispheres and cerebellum were noted. Microscopic examination revealed a neoplastic proliferation of large, atypical lymphoid cells within the lumina of capillaries and small arteries involving all organs examined, including the brain (Fig 1), lung, liver, heart, kidneys (Fig 2), and adrenal glands. This neoplastic lymphoid infiltrate was exclusively intravascular. The malignant cells displayed positive immunohistochemical staining for B-cell marker CD20 (Fig 3) and negative staining for T-cell markers CD3 (Fig 4) and CD34 (human progenitor cell antigen, blast marker). The findings were diagnostic of intravascular large B-cell lymphoma.
Intravascular large B-cell lymphoma was first described in 1959 as "angioendotheliomatosis proliferans systemisata."1 Since then, fewer than 160 cases have been reported. Primary intravascular lymphoma is a multifocal proliferation of pleomorphic large lymphoid cells within small arteries, veins, and capillaries. Most tumors are of B-cell lineage, with only a small percentage of T-cell lineage.2,3 It is a rare, aggressive lymphoma that has unusual clinical and pathologic features. The tumor is recognized by its propensity to proliferate and remain within vascular spaces, without local tissue or vessel wall infiltration. Intravascular large B-cell lymphoma may present over a wide age range, but it has a predilection for patients in their seventh decade. Men are affected as often as women. The most common manifestations are fever of unknown origin, skin rash, and mental status changes. The CNS and skin are the most frequently involved organs, at least initially, but the great majority of patients show multisystem involvement at autopsy.3 Subcutaneous nodules or masses and indurated plaques of variable size are the most frequently reported skin changes. CNS involvement usually manifests as confusing, bizarre, and nonspecific neurologic symptoms, such as progressive dementia, nonlocalizing neurologic deficits, and focal neurologic signs. Infarcts due to involvement of the vessels of the CNS or peripheral nervous system may be seen in up to 50% of cases, accounting for these symptoms. Virtually any organ in the body may be affected. However, organs that typically are involved by lymphoma, such as the lymph nodes and bone marrow, are rarely enlarged or expanded by the neoplastic process.4 The presence of intravascular large B-cell lymphoma is usually not detectable morphologically on examination of peripheral-blood specimens.5 Laboratory findings include anemia, electrolyte disturbances, and manifestations of disordered immune function. Common to all these presentations are the effects of multiple areas of occlusion of capillaries, arterioles, and venules by large, atypical, noncohesive lymphoid cells. These cells usually have round nuclei, vesicular chromatin, multiple prominent nucleoli, and a moderate rim of amphophilic cytoplasm. The reason the lymphoma cells are often confined within vascular spaces is unclear. Most cases of intravascular large B-cell lymphoma are rapidly fatal, and often the diagnosis is not made until autopsy. In recent years, with more widespread recognition of this entity, more patients have been diagnosed antemortem and have been treated with various types of therapy. Intravascular large B-cell lymphoma should be considered in the differential diagnosis of patients who experience interstitial lung disease, dementia, fever of undetermined origin, unexplained skin changes, and renal failure.2 Data suggest that complete remission and possibly long-term survival may be achieved if the diagnosis is prompt and appropriate chemotherapy (directed at intermediate- and high-grade lymphomas) is initiated early.2,6 REFERENCES 1. Pfleger L, Tappeneir J: Zur Kenntnis des systemisierten Endotheliomatose der cutanen Blutgafaesse. Hautarzt 10: 359-363, 1959[Medline] 2. Demirer T, Dail DH, Aboulafin DM, et al: Four varied cases of intravascular lymphomatosis and a literature review. Cancer 73: 1783-1745, 1994 3. Sanna P, Bertoni F, Roggero E, et al: Angiothropic (intravascular) large cell lymphoma: Case report and short discussion of the literature. Tumori 83: 772-775, 1997[Medline] 4. Mann RB: Are there site-specific differences among extranodal aggressive B-cell neoplasms? Am J Clin Pathol 111: S144-S150, 1999 (suppl 1)[Medline] 5. Wick MR, Mills SE: Intravascular lymphomatosis: Clinicopathologic features and differential diagnosis. Semin Diagn Pathol 8: 91-101, 1991[Medline]
6.
Levin KH, Lutz G: Angiotropic large cell lymphoma with peripheral nerve and skeletal muscle involvement. Early diagnosis and treatment. Neurology 47: 1009-1011, 1996
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Copyright © 2001 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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