What are risk factors for primary central nervous system lymphoma (PCNSL) in immunocompromised patients?

Updated: Jan 19, 2018
  • Author: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS; Chief Editor: Stephen A Berman, MD, PhD, MBA  more...
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Answer

Answer

The nature, intensity, and duration of immune suppression are factors in determining the risk of developing PCNSL. [7]

Prolonged glucocorticoid use usually is required (>6 mo).

Patients with AIDS who have low CD4+ counts are at the greatest risk for PCNSL. Patients with AIDS generally have CD4+ counts of fewer than 30 cells/µL.

Virtually all PCNSLs in patients with AIDS express an Epstein-Barr virus (EBV)-related genome. PCNSL is less frequently associated with EBV in patients without AIDS.

The overwhelmingly common risk factor for PCNSL related to human immunodeficiency virus (HIV) infection is intravenous (IV) drug abuse.

Corboy et al reported that 56% of a group of immunocompetent and immunocompromised patients had human herpes virus 8 (HHV-8) in their tumors. This is the same herpes virus that is associated with Kaposi sarcoma and with primary effusion (ie, body-cavity-based lymphomas); however, a direct causal relationship of this herpes virus to any PCNSL has not yet been established. [8] (HHV-8 has been detected in PCNSLs by PCR at low copy number, suggesting that HHV-8 is present in a cell compartment other than the malignant one.)

Gomez-Brouchet et al studied 35 patients (17 with and 18 without AIDS) with PCNSL for the presence of HHV-8 in tumor cells. The antibody LN53, which reacts with the latent nuclear antigen 1 (LNA1) of HHV-8, was used on tissue sections from these patients and in addition, DNA was available for PCR. They found none of the 35 cases contained either DNA sequences or LNA1-positive cells in the tumor cells of PCNSL. [9]


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