원발성 중추신경계 림프종

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원발성 중추신경계 림프종
sella turcica와 시상하부에서 중뇌개로 이어지는 원발성 중추신경계 B-세포 비호즈킨 림프종 뇌 MRI (중앙에 집중적으로 흰 부분).
sella turcica시상하부에서 중뇌개로 이어지는 원발성 중추신경계 B-세포 비호즈킨 림프종MRI (중앙에 집중적으로 흰 부분).
ICD-9 200.5

원발성 중추신경계 림프종 (PCNSL)은 면역기능이 억제된 환자들(일반적으로 AIDS환자)에게서 많이 나타나는 원발성 두개내 종양이다. 소교종 and 원발성 뇌 림프종으로도 알려져 있다.[1] PCNSL은 HIV에 감염으로 발생한 림프종의 약 20% 정도로 나타나고 있다. 다른 형태는 버킷 림프종과 면약아구성 림프종이다. 원발성 CNS 림프종은 면역결핍(AIDS 및 장기이식등의 면역억제) 환자의 Epstein-Barr virus (EBV) 감염과 (> 90%) 깊은 연관이 있으며[2], 특정 나이대에 편중되는 현상은 없다. 진단시 평균 CD4+ 는 ~50/uL이다. 면역저하 환자는 예후가 일반적으로 좋지 않다. 면역적격 환자, 즉 AIDS를 가지고 있지 않고 다른 면형결핍이 없는 환자는, 드물게 EBV 간염나 다른 DNA 바이러스와 연관된 경우가 있다. 면역적격자의 경우, PCNSL는 일반적으로 50~60대의 노인 환자에게 나타난다. 중요한 점은, 면역적격자들의 PCNSL의 발병률이 10년동안 인구 10만명중 2.5명에서 30명으로 증가되고 있다고 보고되는 것이다.[3][4] 면역적격자에게서 나타나는 이 질병의 증가원인은 아직 밝혀지지 않았다.

분류[편집]

대부분의 PCNSL은 비만성 B 세포 비호즈킨 림프종이다..[5][6]

징후 및 증상[편집]

원발성 중추신경계 림프종은 발작, 두통, 뇌신경 이상소견, 성격전환, 또는 종괴효과에 의한 다른 주요 산경학적 손실이 일반적으로 나타난다.[7][8] 전산증상으로는 고열, 밤중 땀흘림, 체중감소가 나타난다. 다른 증상은

이 있다. [9]

진단[편집]

Micrograph showing a primary CNS lymphoma with the characteristic perivascular distribution composed of large cells with prominent nucleoli. Brain biopsy. HPS stain.

The definitive diagnosis is arrived at from tissue, i.e. a biopsy, by a pathologist.

MRI or contrast enhanced 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.

치료[편집]

Surgical resection is usually ineffective because of the depth of the tumour. Treatment with irradiation and corticosteroids often only produces a partial response and tumour recurs in more than 90% of patients. Median survival is 10 to 18 months in immunocompetent patients, and less in those with AIDS. The addition of IV methotrexate and folinic acid (leucovorin) may extend survival to a median of 3.5 years. If radiation is added to methotrexate, median survival time may increase beyond 4 years. However, radiation is not recommended in conjunction with methotrexate because of an increased risk of leukoencephalopathy and dementia in patients older than 60.[10] In AIDS patients, perhaps the most important factor with respect to treatment is the use of highly active anti-retroviral therapy (HAART), which affects the CD4+ lymphocyte population and the level of immunosuppression.[11] The optimal treatment plan for patients with PCNSL has not been determined. Combination chemotherapy and radiotherapy at least doubles survival time, but causes dementia and leukoencephalopathy in at least 50% of patients who undergo it. The most studied chemotheraputic agent in PCNSL is methotrexate (a folate analogue that interferes with DNA repair). Methotrexate therapy in patients with PCNSL typically requires hospitalization for close monitoring and intravenous fluids. Leucovrin is often given for the duration of the therapy. Standard chemotherapeutic regimens for lymphoma such as CHOP are ineffective in PCNSL, probably due to poor penetration of the agents through the blood brain barrier.[11]

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

예후[편집]

면역적격 환자[편집]

방사선 치료의 초기 반응이 가끔은 매우 우수하고, 때른 완전 관해로 나타난다. 그러나, 방사선치료의 반응기간이 짧으며, 치료후 중위 생존기간이 단지 18개월이다. 메소트렉세이트를 기반으로한 화학요법은 일부 연구에서는 메스트렉세이트 화학요법이후의 중위 생존기간이 48개월에 도달할 할 정도로 괄목하게 생존확률이 개선되었다.[12]

AIDS 환자[편집]

Patients with AIDS and PCNSL 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. 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 PCNSL.[12]

주석[편집]

  1. CNS lymphoma, primary information. 《Diseases Database》. 17 April 2011에 확인.
  2. Fine HA, Mayer RJ (December 1993). Primary central nervous system lymphoma. 《Ann. Intern. Med.》 119 (11): 1093–104. PMID 8239229.
  3. 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): 2461–5. PMID 3179963.
  4. 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): 2409–13. PMID 9191531.
  5. Lukes RJ, Collins RD (October 1974). Immunologic characterization of human malignant lymphomas. 《Cancer》 34 (4 Suppl): 1488–503. PMID 4608683.
  6. Jellinger K, Radaskiewicz TH, Slowik F (1975년). Primary malignant lymphomas of the central nervous system in man. 《Acta Neuropathol》 Suppl 6 (Suppl 6): 95–102. PMID 1098380.
  7. Bataille B, Delwail V, Menet E, et al. (February 2000). Primary intracerebral malignant lymphoma: report of 248 cases. 《J. Neurosurg.》 92 (2): 261–6. PMID 10659013. doi:10.3171/jns.2000.92.2.0261.
  8. Herrlinger U, Schabet M, Bitzer M, Petersen D, Krauseneck P (July 1999). Primary central nervous system lymphoma: from clinical presentation to diagnosis. 《J. Neurooncol.》 43 (3): 219–26. PMID 10563426.
  9. Primary CNS Lymphoma at eMedicine
  10. Deangelis LM, Hormigo A (October 2004). Treatment of primary central nervous system lymphoma. 《Semin. Oncol.》 31 (5): 684–92. PMID 15497122. doi:10.1053/j.seminoncol.2004.07.011.
  11. Primary CNS Lymphoma~treatment at eMedicine
  12. Primary CNS Lymphoma~followup at eMedicine

틀:Lymphoid malignancy 틀:Central nervous system tumors 틀:Viral diseases

외부 링크[편집]