primary central nervous system lymphoma

原発性中枢神経系リンパ腫

WordNet

1. (physical chemistry) a sample of matter in which substances in different phases are in equilibrium; "in a static system oil cannot be replaced by water on a surface"; "a system generating hydrogen peroxide"
2. instrumentality that combines interrelated interacting artifacts designed to work as a coherent entity; "he bought a new stereo system"; "the system consists of a motor and a small computer"
3. a group of independent but interrelated elements comprising a unified whole; "a vast system of production and distribution and consumption keep the country going" (同)scheme
4. a complex of methods or rules governing behavior; "they have to operate under a system they oppose"; "that language has a complex system for indicating gender" (同)system of rules
5. a group of physiologically or anatomically related organs or parts; "the body has a system of organs for digestion"
6. a procedure or process for obtaining an objective; "they had to devise a system that did not depend on cooperation"
7. the living body considered as made up of interdependent components forming a unified whole; "exercise helped him get the alcohol out of his system"
8. of first rank or importance or value; direct and immediate rather than secondary; "primary goals"; "a primary effect"; "primary sources"; "a primary interest"
9. a preliminary election where delegates or nominees are chosen (同)primary_election
10. one of the main flight feathers projecting along the outer edge of a birds wing (同)primary feather, primary quill
11. (astronomy) a celestial body (especially a star) relative to other objects in orbit around it
12. not derived from or reducible to something else; basic; "a primary instinct"
13. in or near a center or constituting a center; the inner area; "a central position"
14. a workplace that serves as a telecommunications facility where lines from telephones can be connected together to permit communication (同)telephone exchange, exchange
15. easily agitated; "a nervous addict"; "a nervous thoroughbred"
16. of or relating to the nervous system; "nervous disease"; "neural disorder" (同)neural
17. a neoplasm of lymph tissue that is usually malignant; one of the four major types of cancer

PrepTutorEJDIC

1. 〈C〉(関連した部分から成る)『体系』,系統,組織[網],装置 / 〈C〉(教育・政治などの)『制度』,機構;《the~》体制 / 〈C〉(思想・学問などの)『体系』,学説 / 〈C〉(…の)『方法』,方式,やり方《+of doing》 / 〈U〉正しい方針(筋道,順序) / 〈U〉《the~》(身体の)組織,系統 / 〈U〉《the~,one's~》身体,全身
2. 『第一の』,『主要な』 / 『初期の』,『初等の』,初級の / 『根本的な』,基本的な,本来の,直接的な / 《名詞の前にのみ用いて》(回路・コイル・巻き・電流などが)一次の / 原色(三原色の一つ) / 《米》=primary election
3. 『中心の』,中央の,中心からの / 『主要な』,中心的な(main) / (音声が)中舌音の / 電話交換局(《英》[telephone]exchange)
4. 『神経の』 / 『神経質な』,神経過敏な / 『心配な』,不安な

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/05/29 16:32:53」(JST)

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[Wiki en表示]

Primary central nervous system lymphoma
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).
Classification and external resources
Specialty	Hematology and oncology
ICD-9	200.5
DiseasesDB	29451
eMedicine	article/1157638

A primary central nervous system lymphoma (PCNSL), also known as microglioma and primary brain lymphoma, is a primary intracranial tumor appearing mostly in patients with severe immunosuppression (typically patients with AIDS). 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 iatrogenically immunosuppressed), and does not have a predilection for any particular age group. Mean CD4+ count at time of diagnosis is ~50/uL. In immunocompromised patients, prognosis is usually poor. In immunocompetent patients (that is, patients who do not have AIDS or some other 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 50's and 60's. 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. The cause for the increase in incidence of this disease in the immunocompetent population is unknown.

Classification

Most PCNSLs are diffuse large B cell non-Hodgkin lymphomas.

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. Systemic symptoms may include fever, night sweats, or weight loss.Other symptoms include

• diplopia
• dysphagia
• vertigo
• monocular vision loss
• progressive dementia or stupor in patients with a nonfocal neurologic exam and minimal abnormalities on MRI (more common in AIDS patients)
• facial hypoesthesia

Diagnosis

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.

Treatment

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. 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. 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.

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

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.

In AIDS patients

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.

References

External links

• MedPix Teaching File MR Scans of Primary Brain Lymphoma

UpToDate Contents

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• 1. 原発性中枢神経系リンパ腫の治療および予後 treatment and prognosis of primary central nervous system lymphoma
• 2. 原発性中枢神経系リンパ腫の臨床症状、病理学的特徴、および診断 clinical presentation pathologic features and diagnosis of primary central nervous system lymphoma
• 3. AIDS関連リンパ腫：原発性中枢神経系リンパ腫 aids related lymphomas primary central nervous system lymphoma
• 4. 中枢神経系病変を有するHIV感染患者に対するアプローチ approach to hiv infected patients with central nervous system lesions
• 5. 後十字靱帯損傷 posterior cruciate ligament injury

English Journal

• Development of second primary malignancy in patients with non-Hodgkin lymphoma: a nationwide population-based study.

• Chien SH1, Liu CJ1,2,3, Hong YC2,4, Teng CJ2,5, Hu YW2,6, Ku FC7, Yeh CM3, Chiou TJ2,8, Gau JP9,10, Tzeng CH1,2.
• Journal of cancer research and clinical oncology.J Cancer Res Clin Oncol.2015 Nov;141(11):1995-2004. doi: 10.1007/s00432-015-1979-1. Epub 2015 May 14.
• BACKGROUND: With the improved survival of non-Hodgkin lymphoma (NHL) patients, development of second primary malignancy (SPM) has become an increasingly important issue in these long-term survivors.METHODS: We conducted a retrospective study to analyze NHL patients diagnosed between January 1997 and
• PMID 25971624

• A focus on pharmacotherapy for primary central nervous system lymphoma.

• Korfel A1.
• Expert review of hematology.Expert Rev Hematol.2015 Oct;8(5):559-62. doi: 10.1586/17474086.2015.1066672. Epub 2015 Jul 9.
• The prognosis of primary central nervous system lymphoma (PCNSL) is still poor compared to other diffuse large B-cell lymphomas. High-dose methotrexate-based chemotherapy plays a central role in the management of PCNSL; however, the optimal regimen has not been established thus far. Consolidating wh
• PMID 26159859

• Rituximab with High Dose Methotrexate in Primary Central Nervous System Lymphoma.

• Kansara R1, Shenkier TN1, Connors JM1, Sehn LH1, Savage KJ1, Gerrie AS1,2, Villa D1.
• American journal of hematology.Am J Hematol.2015 Sep 28. doi: 10.1002/ajh.24204. [Epub ahead of print]
• The addition of rituximab (R) to chemotherapy improves outcomes in patients with systemic B-cell non-Hodgkin lymphomas, but the impact in patients with primary central nervous system lymphoma (PCNSL) receiving high-dose methotrexate (HDMTX) is unknown. Patients diagnosed with PCNSL at the British Co
• PMID 26414492

Japanese Journal

• 脳悪性リンパ腫と認知症 (特集 治せる認知症)

• Brain and nerve 68(4), 383-390, 2016-04
• NAID 40020814424

• Clinical Impact of a Humanized CCR4 Antibody (Mogamulizumab) in 14 Patients with Aggressive Adult T-cell Leukemia-lymphoma Treated at a Single Institution During a Three-year Period (2012-2014)

• Internal Medicine 55(11), 1439-1445, 2016
• NAID 130005155327

• 2回目の脳生検により原発性中枢神経系血管炎の確定診断に至り治療しえた1例

• 臨床神経学 56(3), 186-190, 2016
• NAID 130005142030

Related Links

• Primary CNS Lymphoma Treatment (PDQ®) - National Cancer Institute

18 Oct 2012 ... Primary central nervous system (CNS) lymphoma is a disease in which malignant (cancer) cells form in the lymph tissue ... Primary CNS lymphoma can start in the brain, spinal cord, or meninges (the layers that form the outer ...

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★リンクテーブル★

リンク元	「原発性中枢神経系リンパ腫」
関連記事	「primary」「nervous」「central」「system」

「原発性中枢神経系リンパ腫」

　　[★]

英

primary central nervous system lymphoma, primary CNS lymphoma

参考

• http://merckmanual.jp/mmpej/sec14/ch192/ch192b.html#sec14-ch192-ch192b-2729

「primary」

　　[★]

• adj.

• 一次の、第一次の、主要な、(化学)第一級の、一級の、初生の、(病名)原発性の、原発の、プライマリーの

関

cardinal、chief、first-order、foremost、main、mainly、major、master、primarily、principal、principally

　 　 　 　 　 　

「nervous」

　　[★]

• adj.

• 神経の、心配な、神経質な

関

anxiety、anxious、anxiously、care、concern、fear、nerve、nervi、nervousness、nervus、neural、neuro、neuron、neuroticism、trouble、uneasy、worrisome、worry

　 　 　

「central」

　　[★]

• adj.

• 中心の、中心的な、中心性の、中枢的な、中枢性の、中枢の、中枢神経性の

関

center、centrally、centrally acting、centre、centro、centrum、hub、medial、pivotal、pivotally

　 　 　

「system」

　　[★]

• n.

• 系、システム、体系、体制、方式

関

method、series、strain