eMedicine Specialties > Neurology > Neurological Infections

HIV-1 Associated Opportunistic Neoplasms - CNS Lymphoma

Author: Niranjan N Singh, MD, DNB, Assistant Professor of Neurology, University of Missouri Columbia
Coauthor(s): Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Contributor Information and Disclosures

Updated: May 8, 2007

Introduction

Background

HIV-associated CNS lymphoma is a diffuse, large-cell non-Hodgkin lymphoma that usually occurs in the brain (rarely in the spinal cord) and causes focal neurologic signs and mental status changes. It is a late complication of HIV infection.

Pathophysiology

HIV-associated CNS lymphoma is typically of B-cell origin. Almost 100% of affected patients exhibit evidence of Epstein-Barr virus (EBV) in the lymphomatous lesions and the cerebrospinal fluid (CSF). EBV transformation of chronically activated B cells is probably responsible for lymphoma development. Development of this opportunistic neoplasm is associated with CD4+ lymphocyte counts less than 100 cells/mm3.

Frequency

United States

HIV-associated CNS lymphoma is the second most common mass lesion (after toxoplasmosis) in patients with AIDS and occurs in up to 5% of these patients. In up to 0.6% of patients, it is the presenting feature of AIDS. A definite decline in the incidence of HIV-associated CNS lymphoma occurred in the post–highly active antiretroviral therapy (HAART) era.

International

In a retrospective analysis at a German center, the incidence of primary CNS lymphoma peaked at 5.33 per 1000 person-years from 1991-1994 (pre-HAART) and then declined to 0.32 per 1000 person-years after 1999 (post-HAART).1

Mortality/Morbidity

The prognosis of HIV-associated CNS lymphoma has improved with the advent of HAART.

  • In the pre-HAART era, median survival was poor with death occurring a few weeks after diagnosis.
  • In the post-HAART era, the 2-year survival increased to 29%.2
  • In another study, 6 of 7 HAART-treated patients were alive at a median follow-up of 667 days.3

Clinical

History

  • The onset of CNS lymphoma is often more insidious than that of toxoplasmosis.
  • Presenting symptoms may include lethargy, confusion, impaired memory, headache, seizures, or focal weakness.
  • Fever is usually absent.

Physical

  • Lethargy, confusion, impaired memory, and focal neurologic signs may be noted on physical examination.
  • Funduscopy may reveal ocular involvement.

More on HIV-1 Associated Opportunistic Neoplasms - CNS Lymphoma

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Differential Diagnoses & Workup: HIV-1 Associated Opportunistic Neoplasms - CNS Lymphoma
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Follow-up: HIV-1 Associated Opportunistic Neoplasms - CNS Lymphoma
Multimedia: HIV-1 Associated Opportunistic Neoplasms - CNS Lymphoma
References
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References

  1. Wolf T, Brodt HR, Fichtlscherer S. Changing incidence and prognostic factors of survival in AIDS-related non-Hodgkin's lymphoma in the era of highly active antiretroviral therapy (HAART). Leuk Lymphoma. Feb 2005;46(2):207-15. [Medline].

  2. Biggar RJ, Engels EA, Ly S. Survival after cancer diagnosis in persons with AIDS. J Acquir Immune Defic Syndr. Jul 1 2005;39(3):293-9. [Medline].

  3. Skiest DJ, Crosby C. Survival is prolonged by highly active antiretroviral therapy in AIDS patients with primary central nervous system lymphoma. AIDS. Aug 15 2003;17(12):1787-93. [Medline].

  4. Bossolasco S, Falk KI, Ponzoni M. Ganciclovir is associated with low or undetectable Epstein-Barr virus DNA load in cerebrospinal fluid of patients with HIV-related primary central nervous system lymphoma. Clin Infect Dis. Feb 15 2006;42(4):e21-5. [Medline].

  5. Aboulafia DM, Ratner L, Miles SA. Antiviral and immunomodulatory treatment for AIDS-related primary central nervous system lymphoma: AIDS Malignancies Consortium pilot study 019. Clin Lymphoma Myeloma. Mar 2006;6(5):399-402. [Medline].

  6. AAN Quality Standards Subcommittee. Evaluation and management of intracranial mass lesions in AIDS. Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. Jan 1998;50(1):21-6. [Medline].

  7. de Gans J, Portegies P. Neurological complications of infection with human immunodeficiency virus type 1. A review of literature and 241 cases. Clin Neurol Neurosurg. 1989;91(3):199-219. [Medline].

  8. Forsyth PA, DeAngelis LM. Biology and management of AIDS-associated primary CNS lymphomas. Hematol Oncol Clin North Am. Oct 1996;10(5):1125-34. [Medline].

  9. Gendelman HE, Lipton SA, Epstein L. The Neurology of AIDS. New York: Chapman & Hall; 1998.

  10. Hoffman C. Primary CNS lymphoma. In: HIV Medicine. 2005.

  11. Noy A. Update in HIV lymphoma. Curr Opin Oncol. Sep 2006;18(5):449-55. [Medline].

  12. Offiah CE, Turnbull IW. The imaging appearances of intracranial CNS infections in adult HIV and AIDS patients. Clin Radiol. May 2006;61(5):393-401. [Medline].

  13. Said G, Saimont AG, Lacroix C. Neurological Complications of HIV and AIDS. Philadelphia: WB Saunders; 1998.

  14. Singh A, Strobos RJ, Singh BM, et al. Steroid-induced remissions in CNS lymphoma. Neurology. Nov 1982;32(11):1267-71. [Medline].

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Further Reading

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Keywords

Acquired immunodeficiency syndrome, AIDS, HIV-associated CNS lymphoma, Epstein-Barr virus, EBV, EVB infection, toxoplasmosis, large-cell non-Hodgkin lymphoma, large-cell non-Hodgkin's lymphoma, non-Hodgkin's lymphoma, non-Hodgkin lymphoma, late complication of HIV infection, HIV infection

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Contributor Information and Disclosures

Author

Niranjan N Singh, MD, DNB, Assistant Professor of Neurology, University of Missouri Columbia
Niranjan N Singh, MD, DNB is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Coauthor(s)

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

Medical Editor

Ronald A Greenfield, MD, Professor, Department of Internal Medicine, Section of Infectious Diseases, University of Oklahoma College of Medicine
Ronald A Greenfield, MD is a member of the following medical societies: American College of Physicians, American Federation for Medical Research, American Society for Microbiology, Central Society for Clinical Research, Infectious Diseases Society of America, Medical Mycology Society of the Americas, Phi Beta Kappa, Southern Society for Clinical Investigation, and Southwestern Association of Clinical Microbiology
Disclosure: Pfizer Honoraria Speaking and teaching; Gilead Honoraria Speaking and teaching; Ortho McNeil Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Honoraria Speaking and teaching; Astellas Honoraria Speaking and teaching; Cubist  Speaking and teaching

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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