Primary central nervous system lymphoma natural history, complications, and prognosis: Difference between revisions

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*[[Neurotoxicity]] typically is associated with significant cognitive, motor and autonomic dysfunction, and has a negative impact on quality of life.  
*[[Neurotoxicity]] typically is associated with significant cognitive, motor and autonomic dysfunction, and has a negative impact on quality of life.  
*Delayed neurologic toxicity is a serious complication, especially occurring in patients older than 60 years.
*Delayed neurologic toxicity is a serious complication, especially occurring in patients older than 60 years.
*[[Methotrexate|Methotrexate (MTX)]] is a known neurotoxin and has the potential of producing '''leukoencephalopathy''' as well as other types of neurotoxicities, such as microangiopathy. MTX is a folate antagonist inhibiting nucleic acid and methioine synthesis. [[Methionine]] is necessary for CNS myelination. The presence of a risk haplotype defined by polymorphisms influencing methionine metabolism referred a relative risk for CNS white matter changes. MTX in combination with [[radiation|whole brain radiotherapy (WBRT)]] relates to its potential for causing delayed leukoencephalopathy.<ref name="Yamanaka2013">{{cite journal|last1=Yamanaka|first1=Ryuya|title=Primary Central Nervous System Lymphoma − Recent Advance on Clinical Research|year=2013|doi=10.5772/52757}}</ref>


==Prognosis==
==Prognosis==

Revision as of 18:50, 17 April 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Overview

If left untreated, primary central nervous system lymphoma may progress to develop elevated intracranial pressure, ocular symptoms, focal neurological deficits, and neuropsychiatric problems.[1] Common complications of primary central nervous system lymphoma include relapse, extracranial or subcutaneous metastasis, neuropsychiatric problems, and neurological toxicity.[1][2][3] The prognosis of primary central nervous system lymphoma is generally poor.[2][4]

Natural History

  • Primary central nervous system lymphoma is usually a fast-growing (aggressive) lymphoma.[4]
  • Primary central nervous system lymphoma often recurs after treatment.
  • The clinical course is rapidly fatal; these patients usually present with signs of elevated intracranial pressure, nausea, papilledema, vomiting, and neuropsychiatric symptoms.[5]

Complications

Common complications of primary central nervous system lymphoma include:[1][2][3]

  • Neurological toxicity

Neurological Toxicity

  • As survival of patients with primary central nervous system lymphoma becoming long, the quality o life and mental function is now very important.[3]
  • Neurotoxicity typically is associated with significant cognitive, motor and autonomic dysfunction, and has a negative impact on quality of life.
  • Delayed neurologic toxicity is a serious complication, especially occurring in patients older than 60 years.
  • Methotrexate (MTX) is a known neurotoxin and has the potential of producing leukoencephalopathy as well as other types of neurotoxicities, such as microangiopathy. MTX is a folate antagonist inhibiting nucleic acid and methioine synthesis. Methionine is necessary for CNS myelination. The presence of a risk haplotype defined by polymorphisms influencing methionine metabolism referred a relative risk for CNS white matter changes. MTX in combination with whole brain radiotherapy (WBRT) relates to its potential for causing delayed leukoencephalopathy.[3]

Prognosis

  • The prognosis of primary central nervous system lymphoma is generally poor.[4][2]
  • Poor prognostic factors for primary central nervous system lymphoma include:[4][6]

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 is 18 months.
  • Methotrexate-based chemotherapy markedly improves survival, with some studies showing median survival after methotrexate chemotherapy reaching 48 months.[7]

In AIDS patients

  • Patients with AIDS and primary central nervous system lymphoma have a median survival of only 4 months with radiotherapy alone. Untreated, median survival is only 2.5 months, sometimes due to concurrent opportunistic infections rather than the lymphoma itself.[7]
  • Extended survival has been seen, however, in a subgroup of AIDS patients with CD4 counts of more than 200 and no concurrent opportunistic infections, who can tolerate aggressive therapy consisting of either methotrexate monotherapy or vincristine, procarbazine, or whole brain radiotherapy.
  • These patients have a median survival of 10–18 months. Of course, 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 primary CNS lymphoma.[7]

References

  1. 1.0 1.1 1.2 Symptoms of primary CNS Lymphoma. Lymphomation 2016. http://www.lymphomation.org/type-cns.htm. Accessed on February 24, 2016
  2. 2.0 2.1 2.2 2.3 Ahmed, Zartaj; Ramanathan, Ramesh K.; Ram, Sunil; Newell, James; Halepota, Maqbool (2014). "Unusual Relapse of Primary Central Nervous System Lymphoma at Site of Lumbar Puncture". Case Reports in Hematology. 2014: 1–4. doi:10.1155/2014/161952. ISSN 2090-6560.
  3. 3.0 3.1 3.2 3.3 Yamanaka, Ryuya (2013). "Primary Central Nervous System Lymphoma − Recent Advance on Clinical Research". doi:10.5772/52757.
  4. 4.0 4.1 4.2 4.3 Prognosis of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016
  5. Manenti, G.; Di Giuliano, F.; Bindi, A.; Liberto, V.; Funel, V.; Garaci, F. G.; Floris, R.; Simonetti, G. (2013). "A Case of Primary T-Cell Central Nervous System Lymphoma: MR Imaging and MR Spectroscopy Assessment". Case Reports in Radiology. 2013: 1–5. doi:10.1155/2013/916348. ISSN 2090-6862.
  6. Prognsotic factors for primary CNS lymphoma. National cancer institute 2016. http://www.cancer.gov/types/lymphoma/hp/primary-cns-lymphoma-treatment-pdq. Accessed on February 19, 2016
  7. 7.0 7.1 7.2 Prognosis of primary central nervous system lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Primary_central_nervous_system_lymphoma. Accessed on February 18, 2016


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