Immunodeficiency-associated CNS lymphomas

Last revised by Daniel J Bell on 9 May 2023

Immunodeficiency-associated CNS lymphomas are recognized in the WHO classification of CNS tumors (2021, 5th edition) as one of the distinct diagnoses nested within primary CNS lymphomas. Within this diagnosis are a number of entities previously recognized as separate entities, all of which share immunocompromised hosts, large B-cell morphology and are EBV-related.

In previous WHO classifications, and therefore still widely seen in the literature, are a number of specific diagnoses that no longer warrant their own diagnosis but what are, nonetheless, worth discussing individually as they occur in distinct clinical contexts. These include:

Essentially any cause of immunosuppression increases the risk of developing a primary CNS lymphoma. These include 1

As such the epidemiology will reflect those of the underlying condition/cause and will vary from region to region. Overall, immunodeficiency-associated CNS lymphomas account for 8-10% of all lymphomas 2.

Immunodeficiency-associated CNS lymphomas are of B-cell origin and therefore have the usual B-cell markets (CD19, CD20 and CD79a). In most instances, and unlike sporadic primary CNS lymphomas, these tumors are associated with prior EBV infection 1.

They express EBER1, EBER2, LMP1 and EBNA1-6 1.

Although generally, the features of immunodeficiency-associated CNS lymphomas is similar to that of sporadic primary diffuse large B-cell CNS lymphoma, in the setting of immunodeficiency tumors are far more likely to demonstrate 1,2

  • heterogeneous signal

  • peripheral enhancement with central non-enhancement due to necrosis

  • multifocality

  • surrounded by a greater degree of vasogenic edema

It is important to note, however, these findings are far from pathognomonic for immunodeficiency, and are seen, albeit less frequently, in immunocompetent primary diffuse large B-cell lymphoma of the CNS.

The combination of immunosuppression/immunodeficiency and an aggressive tumor, immunodeficiency-associated CNS lymphomas generally carries a poor prognosis 1.

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Cases and figures

  • Figure 1: gross pathology
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  • Case 1
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  • Case 2
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