Primary central nervous system lymphoma information
Medical condition
Primary central nervous system lymphoma
Other names
Microglioma and Primary brain lymphoma[1]
Brain magnetic resonance imaging showing primary central nervous system B-cell non-Hodgkin lymphoma of the sella turcica and hypothalamus, continuing to the tectum (intensely white areas in the middle).
Specialty
Hematology and Neuro-oncology
Primary central nervous system lymphoma (PCNSL), also termed primary diffuse large B-cell lymphoma of the central nervous system (DLBCL-CNS),[2] is a primary intracranial tumor appearing mostly in patients with severe immunodeficiency (typically patients with 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 lymphomas in HIV infections. (Other types are Burkitt's lymphomas and immunoblastic lymphomas). Primary CNS lymphoma is highly associated with 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.
^"CNS lymphoma, primary information". Diseases Database. Retrieved 17 April 2011.
^Grimm KE, O'Malley DP (February 2019). "Aggressive B cell lymphomas in the 2017 revised WHO classification of tumors of hematopoietic and lymphoid tissues". Annals of Diagnostic Pathology. 38: 6–10. doi:10.1016/j.anndiagpath.2018.09.014. PMID 30380402. S2CID 53196244.
^Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ (January 2020). "Diffuse large B-cell lymphoma variants: an update". Pathology. 52 (1): 53–67. doi:10.1016/j.pathol.2019.08.013. PMID 31735345.
^Fine HA, Mayer RJ (December 1993). "Primary central nervous system lymphoma". Annals of Internal Medicine. 119 (11): 1093–1104. doi:10.7326/0003-4819-119-11-199312010-00007. PMID 8239229. S2CID 27535985.
^Eby NL, Grufferman S, Flannelly CM, Schold SC, Vogel FS, Burger PC (December 1988). "Increasing incidence of primary brain lymphoma in the US". Cancer. 62 (11): 2461–2465. doi:10.1002/1097-0142(19881201)62:11<2461::aid-cncr2820621135>3.0.co;2-m. PMID 3179963.
^Corn BW, Marcus SM, Topham A, Hauck W, Curran WJ (June 1997). "Will primary central nervous system lymphoma be the most frequent brain tumor diagnosed in the year 2000?". Cancer. 79 (12): 2409–2413. doi:10.1002/(sici)1097-0142(19970615)79:12<2409::aid-cncr17>3.3.co;2-2. PMID 9191531.
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