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Pathogenesis:
Primary CNS lymphoma often occurs as a late complication of AIDS. Untreated, the survival rate of patients with AIDS-related primary CNS lymphoma is less than 2 months. With high doses of radiation, response rates have improved modestly (Baumgartner et al.), with reports of median survival time of 2 to 5 months. Patients usually die from opportunistic infections. The increased incidence of concurrent systemic and CNS infections (cytomegalovirus, varicella zoster virus, progressive multifocal leukoencephalopathy) in AIDS patients with CNS lymphoma may indicate that viral infections are somehow linked to the pathogensisis of CNS lymphoma in the immunocompromised patient.
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Symptoms:
So et al. report focal neurologic signs such as hemiparesis (Partial paralysis affecting one side of the body) or aphasia (loss of ability to speak or understand language) in 35%, seizures in 15%, and cranial nerve palsies in 10% of people with CNS lymphoma. Confusion, memory loss, and lethargy were reported in 60%.
Gill et al. report changes in personality, apathy and confusion. Sometimes the only manifestation is a headache. Cranial nerve palsies, other than facial nerve paralysis, should be regarded as lymphomatous in origin until proven otherwise. Symptoms are not unlike those of toxoplasmic encephalitis adding further complications in making a confirmed diagnosis.
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Diagnosis:
Definitive diagnosis is usually only possible by brain biopsy. Biopsies are often not performed, however, for a variety of reasons including the overall status of the patient and the reluctance of some neurosurgeons to perform the procedure. Primarily, magnetic resonance imaging (MRI) and computed axial tomography (CAT) scans are used to make a presumptive diagnosis. Lumbar punctures with cytologic examination of the spinal fluid may permit a diagnosis in some cases. CNS lymphoma may present as single or multiple lesions which are sometimes indistinguishable from toxoplasmic encephalitis. If a biopsy cannot be performed and the patient has negative serology tests for toxoplasmosis, radiation therapy should be considered.
Patients unresponsive to radiation should be further evaluated.
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Treatment:
Large dose whole-brain radiation is the preferred method of treatment in patients with AIDS-related primary CNS lymphoma. Corticosteroids such as dexamethasone may be introduced to the treatment plan producing further shrinkage of edema and tumor. Although radiation often results in complete remission (20-50%), relapse is likely and the overall prognosis is poor. The aim of radiotherapy may not be towards greater survival time but rather an improvement in the quality of life. The role of chemotherapy (both systemic and intrathecal) prior to and following whole-brain radiation therapy is under investigation; this approach has produced improved results in patients with primary CNS lymphoma unrelated to HIV infection.
A clinical trial combining CHOD, G-CSF, radiotherapy, and cytarabine (ACTG 252) is currently underway for the treatment of CNS lymphoma. A second trial combining etoposide and cisplatin is also enrolling.
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REFERENCES:
Cesarman E et al. Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS related body-cavity-based lymphomas. NEJM 332:1181-5, 1995.
Gill PS et al. HIV-related malignant lymphoma: clinical aspects, treatment, and pathogenesis. Canc Invest 6: 413-6, 1988.
Hessol NA et al. Increased incidence of Hodgkin disease in homosexual men with HIV infection. Ann Int Med 117:309-11, 1992.
Hernier BG at al. Pathogenesis of AIDS lymphomas. AIDS 8: 1025-1049, 1994.
MacMahon EME et al. Epstein-Barr virus in AIDS-related primary central nervous system lymphoma. Lancet 338: 969-73, 1991.
Schiramizu B et al. Identification of a common clonal human immunodeficiency virus integration site in human immunodeficiency virus-associated lymphomas. Cancer Res
54: 2069-72,1994.
So Y et al. Primary central nervous system lymphoma in AIDS: a clinical and pathological study. Ann Neurol 20: 566-72, 1986.
OTHER REPORTS:
Galleto G and Levine A. AIDS-associated primary central nervous system lymphoma (commentary). JAMA 269:92-3, 1993.
Leven A et al. Multicenter phase II study of mitoguazone (MGBG) in relapsed or refractory AIDS-lymphoma. Proc Am Soc Clin Oncol, 1995.
Levine A. Lymphoma in acquired immunodeficiency syndrome. Sem in Oncol 17(l):
104-112,1990.
Levine A et al. Low-dose chemotherapy with central nervous system prophylaxis and zidovudine maintenance in AIDS-related lymphoma. JAMA 266: 84-8, 1991.
Marco M. The lymphoma project report: current issues in research and treatment of AIDS associated lymphoma. Treatment Action Group, 1995.
Remick SC et al. Novel oral combination chemotherapy in the treatment of intermediate-grade and high-grade AIDS-related non-Hodgkin's lymphomas. J Clin Onc 11: 1691-1701, 1993.
Urba WJ and Longo DL. Hodgkin's disease (review article). NEJM 326: 678-87, 1992.
Baumgartner J et al. Primary central nervous system lymphoma: Natural history and response to radiation therapy in 55 patents with acquired immunodeficiency syndrome. J Neurosurg 73:206-211, 1990.
Canellos GP et al. Chemotherapy of advanced Hodgkin's disease with MOPP, ABVD, or MOPP alternating with ABVD. NEJM 327:1478-84,1992.
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