Central Nervous System Lymphoma in HIV Workup
- Author: Florian P Thomas, MD, PhD, Drmed, MA, MS; Chief Editor: Niranjan N Singh, MD, DM more...
Almost 100% of affected patients exhibit evidence of Epstein-Barr virus (EBV) in the CSF and lymphomatous lesions. The development of HIV-associated CNS lymphoma is typically associated with a CD4+ lymphocyte count below 100 cells/mL. Chest radiography and an abdominal ultrasound may be indicated to rule out systemic lymphoma as the underlying cause.
CSF analysis in patients with CNS lymphoma shows pleocytosis and elevated protein. Cytologic results are positive for monoclonal malignant-appearing lymphocytes.
Amplification of EBV deoxyribonucleic acid (DNA) in CSF using the polymerase chain reaction (PCR) corroborates the diagnosis of primary CNS lymphoma. However, the declining incidence of CNS lymphoma may be diminishing the specificity of this finding. Quantitative PCR may increase specificity: Corcoran et al found that using a cut-off of 10,000 EBV DNA copies/mL improved the specificity and positive predictive value when compared with a qualitative result for the diagnosis of CNS lymphoma (96% vs 66%, and 50% vs 10%, respectively). The EBV DNA in the CSF has a low positive predictive value (10-50%) when used in isolation as a positive marker because elevated EBV DNA is also seen in patients with HIV without primary CNS lymphoma.
On computed tomography scans, a hypodense or hyperdense lesion that enhances in a nodular, homogeneous, or ringlike pattern may be observed. Significant edema and mass effect may be present. (See the image below.)
Multiple lesions can occur, although less frequently than with toxoplasmosis. Magnetic resonance imaging (MRI) may reveal additional lesions.
A thallium-201 single-photon emission computed tomography (201 TI SPECT) scan may be useful in distinguishing between lymphoma and toxoplasmosis. Increased201 Tl uptake co-localizing with the lesion on MRI is highly specific for primary CNS lymphoma.
Tumor size of at least 2 cm increases the diagnostic yield. Positive results need to be confirmed by biopsy of the identified lesion.
An 18-fluorodeoxyglucose positron emission tomography (18FDG-PET) scan study has a predictive value similar to that of201 TI SPECT.
MR spectroscopy in primary CNS lymphoma typically shows decreased N -acetylaspartate and creatine, increased choline (suggesting a tumoral cell proliferation), and at-baseline lipid-lactate peaks reflecting necrosis, which is a common feature of primary CNS lymphoma.
Definitive diagnosis requires stereotactic brain biopsy, usually after a therapeutic trial for cerebral toxoplasmosis. Histologic findings in CNS lymphoma vary and consist of a small, noncleaved type and a large, immunoblastic type. (See the image below.)
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