Primary central nervous system lymphoma

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Primary central nervous system lymphoma
Other namesMicroglioma and Primary brain lymphoma
tectum (intensely white areas in the middle).
SpecialtyHematology and Neuro-oncology

Primary central nervous system lymphoma (PCNSL), also termed primary diffuse large B-cell lymphoma of the central nervous system (DLBCL-CNS),

AIDS). It is a subtype and one of the most aggressive of the diffuse large B-cell lymphomas.[3]

PCNSLs represent around 20% of all cases of

Epstein-Barr virus (EBV) infection (> 90%) in immunodeficient patients (such as those with AIDS and those immunosuppressed),[4] and does not have a predilection for any particular age group. Mean CD4+ count at time of diagnosis is ~50/µL. In immunocompromised patients, prognosis is usually poor. In immunocompetent patients (that is, patients who do not have AIDS or some other acquired or secondary immunodeficiency), there is rarely an association with EBV infection or other DNA viruses
. In the immunocompetent population, PCNSLs typically appear in older patients in their 50s and 60s.

Importantly, the incidence of PCNSL in the immunocompetent population has been reported to have increased more than 10-fold from 2.5 cases to 30 cases per 10 million population.[5][6] The cause for the increase in incidence of this disease in the immunocompetent population is unknown.

Signs and symptoms

A primary CNS lymphoma usually presents with seizure, headache, cranial nerve findings, altered mental status, or other focal neurological deficits typical of a mass effect.[7][8] Systemic symptoms may include fever, night sweats, or weight loss. Other symptoms include

Diagnosis

nucleoli. Brain biopsy. HPS stain
.

The current standard for diagnosis typically includes positive CSF cytology, vitreous biopsy, or brain/leptomeningeal biopsy.[10] Histopathological confirmation is essential for definitive diagnosis.[11]

CT classically shows multiple ring-enhancing lesions in the deep white matter. The major differential diagnosis (based on imaging) is cerebral toxoplasmosis, which is also prevalent in AIDS patients and also presents with a ring-enhanced lesion, although toxoplasmosis generally presents with more lesions and the contrast enhancement is typically more pronounced. Imaging techniques cannot distinguish the two conditions with certainty, and cannot exclude other diagnoses. Thus, patients undergo a brain biopsy or vitreous biopsy, if there is intraocular involvement.[10]

Classification

Most PCNSLs are diffuse large B cell non-Hodgkin lymphomas.[12][13]

Treatment

Surgical

blood brain barrier.[15]

Newer treatments, such as high dose chemotherapy combined with autologous stem cell transplant are proving to increase survival by years.

blood brain barrier with NGR-hTNF followed by CHOP, produced responses in 75% cases. [17]

A phase 1 clinical trial of ibrutinib – an inhibitor of Bruton's tyrosine kinase – in 13 patients reported responses in 10 (77%).[18] Five of the responses were complete.

Prognosis

In immunocompetent patients

The initial response to radiotherapy is often excellent, and may result in a complete remission. However, the duration of response with radiotherapy alone remains short, with median survival after treatment with radiotherapy just 18 months. Methotrexate based chemotherapy markedly improves survival, with some studies showing median survival after methotrexate chemotherapy reaching 48 months.[15]

In AIDS patients

Patients with

highly active antiretroviral therapy (HAART) is critical for prolonged survival in any AIDS patient, so compliance with HAART may play a role in survival in patients with concurrent AIDS and PCNSL.[15]

References

  1. ^ "CNS lymphoma, primary information". Diseases Database. Retrieved 17 April 2011.
  2. S2CID 53196244
    .
  3. PMID 31735345
    .
  4. S2CID 27535985
    .
  5. PMID 3179963
    .
  6. PMID 9191531
    .
  7. PMID 10659013
    .
  8. S2CID 5990227
    .
  9. ^ Primary CNS Lymphoma at eMedicine
  10. ^
    PMID 23319132
    .
  11. PMID 29999484
    .
  12. S2CID 9652828
    .
  13. PMID 1098380
    .
  14. PMID 15497122
    .
  15. ^ a b c d Primary CNS Lymphoma~treatment at eMedicine
  16. PMID 25568347
    .
  17. S2CID 162182856
    .
  18. PMID 28619981
    .

External links
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