WordNet

regulate housing in; of certain areas of towns (同)district
any of the regions of the surface of the Earth loosely divided according to latitude or longitude (同)geographical zone
(anatomy) any encircling or beltlike structure (同)zona
a locally circumscribed place characterized by some distinctive features
an area or region distinguished from adjacent parts by a distinctive feature or characteristic
the boundary line or the area immediately inside the boundary (同)border, perimeter
the amount of collateral a customer deposits with a broker when borrowing from the broker to buy securities (同)security deposit
an amount beyond the minimum necessary; "the margin of victory"
the blank space that surrounds the text on a page; "he jotted a note in the margin"
producing at a rate that barely covers production costs; "marginal industries"
dividing an area into zones or sections reserved for different purposes such as residence and business and manufacturing etc
of or relating to the spleen (同)splenetic, lienal

PrepTutorEJDIC

(何らかの目的・特徴などによって他の地域と区別された)『地帯』,地域,地区(belt, area) / (気候によって地球を大別した)帯(たい) / 《米》(交通・郵便などの)同一料金区域;(郵便番号で分けた)郵便区 / (環状の)ベルト,輪,帯 / …‘を'地帯(地区)に分ける / (…の目的に)〈地域〉‘を'区画規制する《+名+as(for)+名》 / …‘を'帯で巻く,‘に'帯印をつける
『へり』,『縁』,端(edge) / (ページの)『余白』,欄外 / (可能の)限界,限度 / (空間・時間・経費・活動などの)余裕,余地 / 利ざや,マージン,販売利益
縁の,へりの / 限界の,ぎりぎりの,かろうじて要件を満たす / 《名詞の前にのみ用いて》余白に書いた,欄外の / かろうじて帳じりの合う,限界収益点の / 取るに足りない,小さな
(都市計画の)区画制,地区制
脾臓(ひぞう)の,脾臓を冒す

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/04/19 15:57:05」(JST)

wiki en

Splenic marginal zone lymphoma (SMZL) is a lymphoma made up of B-cells that replace the normal architecture of the white pulp of the spleen. The neoplastic cells are both small lymphocytes and larger, transformed blasts, and they invade the mantle zone of splenic follicles and erode the marginal zone, ultimately invading the red pulp of the spleen. Frequently, the bone marrow and splenic hilar lymph nodes are involved along with the peripheral blood. The neoplastic cells circulating in the peripheral blood are termed villous lymphocytes due to their characteristic appearance.^[1]

Synonyms

Under older classification systems, the following names were used:^[1]

Classification system	Name
Rappaport	well-differentiated lymphocytic lymphoma
Lukes-Collins	small lymphocytic lymphoma
Working Formulation	small lymphocytic lymphoma
FAB	splenic lymphoma with circulating villous lymphocytes

Cause

The cell of origin is postulated to be a post-germinal center B-cell with an unknown degree of differentiation.^[1]

Diagnosis

With splenic involvement a requirement for a diagnosis of SMZL, splenomegaly is seen in almost all patients, commonly without lymphadenopathy.^[1] Aside from the uniform involvement of the spleen, the bone marrow is frequently positive in patients with SMZL. Nodal and extranodal involvement are rare.^[1]

Circulating lymphoma cells are sometimes present in peripheral blood, and they occasionally show short villi at the poles of cells and plasmacytoid differentiation.^[2]

Autoimmune thrombocytopenia and anemia sometimes seen in patients with SMZL. Circulating villous lymphocytes are sometimes observed in peripheral blood samples.^[1] A monoclonal paraprotein is detected in a third of patients without hypergammaglobulinemia or hyperviscosity.^[3]^[4]

Reactive germinal centers in splenic white pulp are replaced by small neoplastic lymphocytes that efface the mantle zone and ultimately blend in with the marginal zone with occasional larger neoplastic cells that resemble blasts.^[4]^[5] The red pulp is always involved, with both nodules of larger neoplastic cells and sheets of the small neoplastic lymphocytes. Other features that may be seen include sinus invasion, epithelial histocytes, and plasmacytic differentiation of neoplastic cells.

Involved hilar lymph nodes adjacent to the spleen show an effaced architecture without preservation of the marginal zone seen in the spleen.^[1]

SMZL in bone marrow displays a nodular pattern with morphology similar to what is observed in the splenic hilar lymph nodes.^[6]

Prognosis

Three-quarters of patients survive five or more years; more than half of patients with SMZL survive more than a decade after diagnosis.^[7]

Patients who have a hemoglobin level of less than 12 g/dL, a lactate dehydrogenase level higher than normal, and/or a blood serum albumin levels of less than 3.5 g/dL are likely to have more a aggressive disease course and a shorter survival.^[7] However, even high-risk patients have even odds of living for five years after diagnosis.^[7]

Some genetic mutations, such as mutations in NOTCH2, are also correlated with shorter survival.

Molecular findings

Immunophenotype

The relevant markers that define the immunophenotype for SMZL are shown in the table to the right.^[8] ^[9] The lack of CD5 expression is helpful in the discrimination between SMZL and chronic lymphocytic leukemia/small lymphocytic lymphoma, and the lack of CD10 expression argues against follicular lymphoma. Mantle cell lymphoma is excluded due to the lack of CD5 and cyclin-D1 expression.^[10]

Genetics

Clonal rearrangements of the immunoglobulin genes (heavy and light chains) are frequently seen.^[11] The deletion 7q21-32 is seen in 40% of SMZL patients, and translocations of the CDK6 gene located at 7q21 have also been reported.^[12]

Epidemiology

Less than 1% of all lymphomas are splenic marginal zone lymphomas^[13] and it is postulated that SMZL may represent a large fraction of unclasssifiable CD5- chronic lymphocytic leukemias.^[1] The typical patient is over the age of 50, and gender preference has been described.^[3]

References

^ ^a ^b ^c ^d ^e ^f ^g ^h Elaine Sarkin Jaffe, Nancy Lee Harris, World Health Organization, International Agency for Research on Cancer, Harald Stein, J.W. Vardiman (2001). Pathology and genetics of tumours of haematopoietic and lymphoid tissues. World Health Organization Classification of Tumors 3. Lyon: IARC Press. ISBN 92-832-2411-6.
^ Melo JV, Hegde U, Parreira A, Thompson I, Lampert IA, Catovsky D (June 1987). "Splenic B cell lymphoma with circulating villous lymphocytes: differential diagnosis of B cell leukaemias with large spleens". J. Clin. Pathol. 40 (6): 642–51. doi:10.1136/jcp.40.6.642. PMC 1141055. PMID 3497180.
^ ^a ^b Berger F, Felman P, Thieblemont C et al. (March 2000). "Non-MALT marginal zone B-cell lymphomas: a description of clinical presentation and outcome in 124 patients". Blood 95 (6): 1950–6. PMID 10706860.
^ ^a ^b Mollejo M, Menárguez J, Lloret E et al. (October 1995). "Splenic marginal zone lymphoma: a distinctive type of low-grade B-cell lymphoma. A clinicopathological study of 13 cases". Am. J. Surg. Pathol. 19 (10): 1146–57. PMID 7573673.
^ Jaffe ES, Costa J, Fauci AS, Cossman J, Tsokos M (November 1983). "Malignant lymphoma and erythrophagocytosis simulating malignant histiocytosis". Am. J. Med. 75 (5): 741–9. doi:10.1016/0002-9343(83)90402-3. PMID 6638043.
^ Franco V, Florena AM, Campesi G (December 1996). "Intrasinusoidal bone marrow infiltration: a possible hallmark of splenic lymphoma". Histopathology 29 (6): 571–5. doi:10.1046/j.1365-2559.1996.d01-536.x. PMID 8971565.
^ ^a ^b ^c Arcaini, L. (2006). "Splenic marginal zone lymphoma: a prognostic model for clinical use". Blood 107 (12): 4643–4649. doi:10.1182/blood-2005-11-4659. ISSN 0006-4971.
^ Isaacson PG, Matutes E, Burke M, Catovsky D (1 December 1994). "The histopathology of splenic lymphoma with villous lymphocytes". Blood 84 (11): 3828–34. PMID 7949139.
^ Matutes E, Morilla R, Owusu-Ankomah K, Houlihan A, Catovsky D (15 March 1994). "The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B-cell disorders". Blood 83 (6): 1558–62. PMID 8123845.
^ Savilo E, Campo E, Mollejo M et al. (July 1998). "Absence of cyclin D1 protein expression in splenic marginal zone lymphoma". Mod. Pathol. 11 (7): 601–6. PMID 9688179.
^ Dunn-Walters DK, Boursier L, Spencer J, Isaacson PG (June 1998). "Analysis of immunoglobulin genes in splenic marginal zone lymphoma suggests ongoing mutation". Hum. Pathol. 29 (6): 585–93. doi:10.1016/S0046-8177(98)80007-5. PMID 9635678.
^ Corcoran MM, Mould SJ, Orchard JA et al. (November 1999). "Dysregulation of cyclin dependent kinase 6 expression in splenic marginal zone lymphoma through chromosome 7q translocations". Oncogene 18 (46): 6271–7. doi:10.1038/sj.onc.1203033. PMID 10597225.
^ Armitage JO, Weisenburger DD (August 1998). "New approach to classifying non-Hodgkin's lymphomas: clinical features of the major histologic subtypes. Non-Hodgkin's Lymphoma Classification Project". J. Clin. Oncol. 16 (8): 2780–95. PMID 9704731.

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 脾辺縁帯リンパ腫の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of splenic marginal zone lymphoma
2. 辺縁帯（MALT）リンパ腫の治療 treatment of marginal zone malt lymphoma
3. 肝脾T細胞性リンパ腫の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of hepatosplenic t cell lymphoma
4. リンパ形質細胞性リンパ腫の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of lymphoplasmacytic lymphoma
5. 節外（MALT）および節性濾胞辺縁帯リンパ腫の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of extranodal malt and nodal marginal zone lymphomas

English Journal

Rare presentation of secondary cutaneous involvement by splenic marginal zone lymphoma: report of a case and review of the literature.

Cohen JM1, Nazarian RM, Ferry JA, Takvorian RW, Carter JB.
The American Journal of dermatopathology.Am J Dermatopathol.2015 Jan;37(1):e1-4. doi: 10.1097/DAD.0000000000000055.
: Cutaneous lymphomas encompass a broad spectrum of malignancies, including both primary and secondary cutaneous lymphomas. Determining the exact subtype of cutaneous lymphoma offers prognostic importance and directs therapeutic decisions. We describe the case of a 67-year-old woman with cutaneous i
PMID 25238446

Rapid clearance of HCV-related splenic marginal zone lymphoma under an interferon-free, NS3/NS4A inhibitor-based treatment. A case report.

Rossotti R1, Travi G2, Pazzi A2, Baiguera C2, Morra E3, Puoti M2.
Journal of hepatology.J Hepatol.2015 Jan;62(1):234-7. doi: 10.1016/j.jhep.2014.09.031. Epub 2014 Oct 5.
Chronic infection with hepatitis C virus (HCV) may lead to B cell activation and transformation into non-Hodgkin lymphoma (NHL). Molecular mechanisms of B cell transformation by HCV are poorly understood. One of the most common lymphoproliferative disorders in HCV-infected patients is splenic margin
PMID 25285757

Non-Hodgkin Lymphoma and Hepatitis C: Where We are and What Next?

Sulyok M1, Makara M, Újhelyi E, Vályi-Nagy I.
Pathology oncology research : POR.Pathol Oncol Res.2015 Jan;21(1):1-7. doi: 10.1007/s12253-014-9845-z. Epub 2014 Oct 2.
The association between hepatitis C virus and certain B-cell non-Hodgkin lymphomas, such as marginal zone lymphomas, is supported by epidemiological studies. The exact pathogenetic mechanism is still unknown but both chronic antigenic stimulation and viral lymphotropism may contribute to the evoluti
PMID 25273531

Japanese Journal

寒冷凝集素症を合併し，リツキシマブが奏効した脾臓辺縁帯リンパ腫

安山雅子,川内喜代隆,大塚邦明 [他],田村博之,藤林真理子
日本老年医学会雑誌 51(6), 569-575, 2014
81歳男性．息切れを主訴に来院．末梢血でHb 7.0 g/dL，網状赤血球6.4%，リンパ球20,003/μL, I.Bil 3.6 mg/dL，直接クームス陽性，寒冷凝集素8,192倍，著明な脾腫を認め，PET-CTで脾臓，骨髄に異常集積を認めた．骨髄穿刺及び生検で異型性のあるリンパ球の集簇を認め，表面マーカー解析ではCD5－，CD10－，CD20＋，κ+で免疫染色上cyclin D1－であった …
NAID 130004917187

術前診断しえた脾辺縁帯リンパ腫の1切除例

山下浩正,湯浅典博,竹内英司,後藤康友,三宅秀夫,永井英雅,服部正興,宮田完志,小山大輔,藤野雅彦
日本消化器外科学会雑誌 47(9), 531-537, 2014
　症例は56歳の男性で，2011年1月，腹部膨満・上腹部痛を主訴に当院を受診した．左上腹部に脾腫を触知し血液検査で白血球数増加，血小板数減少，可溶性IL-2受容体の上昇を認めた．腹部CTで脾腫を認め，またFDG-PETでは脾にびまん性高集積を認めた．末梢血・骨髄塗抹標本で細胞質辺縁に不規則な突起を有するリンパ球を認めた．末梢血のフローサイトメトリーによる細胞表面マーカーはCD20（+），CD5（– …
NAID 130004691690

Related Pictures

★リンクテーブル★

リンク元	「脾辺縁帯リンパ腫」「脾性辺縁帯リンパ腫」「脾臓周辺帯リンパ腫」
関連記事	「margin」「zone」「marginal」「splenic」