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2013年09月06日

(2013-09-06 09:51:24)
This article is about lymphoma in humans. For the disease in dogs, cats, and ferrets, seelymphoma in animals.
Lymphoma
Classification and external resources
http://upload.wikimedia.org/wikipedia/commons/thumb/9/9d/Lymphoma_macro.jpg/460px-Lymphoma_macro.jpg
ICD-10 C81-C96
ICD-9 202.8
ICD-O: 9590-9999
MedlinePlus 000580 000581
MeSH D008223

Lymphoma is a type of blood cancer that occurs when B or T lymphocytes,[1] the white blood cells that form a part of the immune system and help protect the body frominfection and disease, divide faster than normal cells or live longer than they are supposed to. Lymphoma may develop in thelymph nodesspleenbone marrow, blood or other organs[2] and eventually they form atumor.[1]

Typically, lymphoma presents as a solidtumor of lymphoid cells. Treatment might involve chemotherapy and in some casesradiotherapy and/or bone marrow transplantation, and lymphomas can be curable depending on the histology, type, and stage of the disease.[3] These malignant cells often originate in lymph nodes, presenting as an enlargement of the node (a tumor). It can also affect other organs in which case it is referred to as extranodal lymphoma. Extranodal sites include the skin, brain, bowels and bone. Lymphomas are closely related to lymphoid leukemias, which also originate in lymphocytes but typically involve only circulating blood and the bone marrow(where blood cells are generated in a process termed haematopoesis) and do not usually form static tumors.[3] There are many types of lymphomas, and in turn, lymphomas are a part of the broad group of diseases calledhematological neoplasms.

EditSigns and symptoms

Lymphoma presents with certain non-specific symptoms. If symptoms are persistent, lymphoma needs to be excluded medically.

EditDiagnosis

Lymphoma is definitively diagnosed by alymph node biopsy, meaning a partial or total excision of a lymph node that is then examined under the microscope.[5] This examination reveals histopathologicalfeatures that may indicate lymphoma. After lymphoma is diagnosed, a variety of tests may be carried out to look for specific features characteristic of different types of lymphoma. These include:

Several classification systems have existed for lymphoma. These systems use histological findings and other findings to divide lymphoma into different categories. The classification of lymphoma can affect treatment and prognosis. Classification systems generally classify lymphoma according to:

  • Whether or not it is a Hodgkin lymphoma.
  • Whether the cell that is replicating is a T cell or B cell.
  • The site that the cell arises from.

Hodgkin's lymphoma

Main article: Hodgkin's lymphoma

Hodgkin's lymphoma is one of the most well-known types of lymphoma,[citation needed] and differs from other forms of lymphoma in itsprognosis and several pathologicalcharacteristics. A division into Hodgkin's and non-Hodgkin's lymphomas is used in several formal classification systems. A Hodgkin's lymphoma is marked by the presence of a type of cell called the Reed-Sternberg cell.[6][7]

WHO classification

The current accepted definition is the WHO classification, published in 2001 and updated in 2008,[8][9] is the latest classification of lymphoma and is based upon the foundations laid within the "Revised European-American Lymphoma classification" (REAL). This system attempts to group lymphomas by cell type (i.e. the normal cell type that most resembles the tumor) and definingphenotypicmolecular or cytogeneticcharacteristics. There are three large groups: the B cellT cell, and natural killer cell tumors. Other less common groups, are also recognized. Hodgkin lymphoma, although considered separately within the World Health Organization (and preceding) classifications, is now recognized as being a tumor of, albeit markedly abnormal, lymphocytes of mature B cell lineage.

Working Formulation

Main article: Working Formulation

The 1996 Working Formulation was a classification of non-Hodgkin lymphoma. It excluded the Hodgkin lymphomas and divided the remaining lymphomas into four grades (Low, Intermediate, High, and Miscellaneous) related to prognosis, with some further subdivisions based on the size and shape of affected cells. This purely histological classification included no information about cell surface markers, or genetics, and it made no distinction betweenT-cell lymphomas or B-cell lymphomas. It was widely accepted at the time of its publication but is now obsolete.[10] It is still used by some cancer agencies for compilation of lymphoma statistics and historical rate comparisons.[citation needed]

Other

  • REAL. In the mid 1990s, the Revised European-American Lymphoma (REAL) Classification attempted to apply immunophenotypic and genetic features in identifying distinct clinicopathologic entities among all the lymphomas except Hodgkin's lymphoma.[11]
  • ICD-O (codes 9590-9999, details at [2])
  • ICD-10 (codes C81-C96, details at [3])

Subtypes

There are many forms of lymphoma. Some forms of lymphoma are categorized as indolent (e.g. small lymphocytic lymphoma), compatible with a long life even without treatment, whereas other forms are aggressive (e.g. Burkitt's lymphoma), causing rapid deterioration and death. However, most of the aggressive lymphomas respond well to treatment and are curable. The prognosistherefore depends on the correct diagnosis and classification of the disease, which is established after examination of a biopsy by apathologist (usually a hematopathologist).[12]

Lymphoma subtypes.[13]
Lymphoma type Relativeincidence[13] Histopathology[13] Immunophenotype Overall
5-year
survival
Other comments [13]
Precursor T-cell leukemia/lymphoma 40% of lymphomas in childhood. Lymphoblastswith irregular nuclear contours, condensed chromatin, small nucleoli and scant cytoplasm without granules. TdTCD2CD7 It often presents as amediastinal massbecause of involvement of thethymus. It is highly associated withNOTCH1 mutations. Most common inadolescent males.
Follicular lymphoma 40% of lymphomas in adults Small "cleaved" cells (centrocytes) mixed with large activated cells (centroblasts). Usually nodular ("follicular") growth pattern CD10, surface Ig 72–77%[14] Occurs in older adults. Usually involves lymph nodes, bone marrow and spleen. Associated with t(14;18) translocationoverexpressing Bcl-2.Indolent
Diffuse large B cell lymphoma 40 to 50% of lymphomas in adults Variable. Most resemble B cells of large germinal centers. Diffuse growth pattern. Variable expression ofCD10 and surface Ig 60%[15] Occurs in all ages, but most commonly in older adults. Often occurs outside lymph nodes. Aggressive.
Mantle cell lymphoma 3 to 4% of lymphomas in adults Lymphocytes of small to intermediate size growing in diffuse pattern CD5 50%[16]to 70%[16] Occurs mainly in adult males. Usually involves lymph nodes, bone marrow, spleen and GI tract. Associated with t(11;14) translocation overexpressing cyclin D1. Moderately aggressive.
B-cell chronic lymphocytic leukemia/lymphoma 3 to 4% of lymphomas in adults Small resting lymphocytes mixed with variable number of large activated cells. Lymph nodes are diffuselyeffaced CD5, surfaceimmunoglobulin 50%.[17] Occurs in older adults. Usually involves lymph nodes, bone marrow and spleen. Most patients have peripheral blood involvement.Indolent.
MALT lymphoma ~5% of lymphomas in adults Variable cell size and differentiation. 40% showplasma celldifferentiation.Homing of B cells to epithelium creates lymphoepithelial lesions. CD5CD10, surface Ig Frequently occurs outside lymph nodes. Very indolent. May be cured by local excision.
Burkitt's lymphoma < 1% of lymphomas in the United States Round lymphoid cells of intermediate size with several nucleoli. Starry-sky appearanceby diffuse spread with interspersedapoptosis. CD10, surface Ig 50%[18] Endemic in Africa, sporadic elsewhere. More common in immunocompromised and in children. Often visceral involvement. Highly aggressive.
Mycosis fungoides Most common cutaneous lymphoid malignancy Usually small lymphoid cells with convoluted nuclei that often infiltrate the epidermis, creating Pautier microabscesses. CD4 75%[19] Localized or more generalized skin symptoms. Generally indolent. In a more aggressive variant,Sézary's disease, there is skinerythema and peripheral blood involvement.
Peripheral T-cell lymphoma-Not-Otherwise-Specified Most common T cell lymphoma Variable. Usually a mix small to large lymphoid cells with irregular nuclear contours. CD3 Probably consists of several rare tumor types. It is often disseminated and generally aggressive.
Nodular sclerosisform of Hodgkin lymphoma Most common type of Hodgkin's lymphoma Reed-Sternberg cell variants and inflammation. usually broad sclerotic bands that consists of collagen. CD15CD30 Most common in young adults. It often arises in themediastinum orcervical lymph nodes.
Mixed-cellularity subtype of Hodgkin lymphoma Second most common form of Hodgkin's lymphoma Many classic Reed-Sternberg cells and inflammation CD15, CD30 Most common in men. More likely to be diagnosed at advanced stages than the nodular sclerosis form.Epstein-Barr virusinvolved in 70% of cases.

Staging

EditTreatment

Prognosis and treatment is different for HL and between all the different forms of NHL,[20]and also depends on the grade of tumour, referring to how quickly a cancer replicates. Paradoxically, high-grade lymphomas are more readily treated and have better prognoses[citation needed]. A well-known example of a high-grade tumour is that ofBurkitt's lymphoma, which is a high-grade tumour that has been known to double within days, but is readily treated.

Low-grade lymphomas

Many low-grade lymphomas remain indolent for many years. In these lymphomas,metastases are very likely. For this reason, treatment of the non-symptomatic patient is often avoided. In these forms of lymphoma[which?]watchful waiting is often the initial course of action. This is carried out because the harms and risks of treatment outweigh the benefits.[21] If a low-grade lymphoma is becoming symptomatic, radiotherapy or chemotherapy are the treatments of choice; although they do not cure the lymphoma, they can alleviate the symptoms, particularly painfullymphadenopathy. Patients with these types of lymphoma can live near-normal lifespans, but the disease is incurable.

High-grade lymphomas

Treatment of some other, more aggressive, forms of lymphoma[which?] can result in a cure in the majority of cases, but the prognosis for patients with a poor response to therapy is worse.[22] Treatment for these types of lymphoma typically consists of aggressive chemotherapy, including the CHOP or R-CHOP regimen.

Hodgkin lymphoma typically is treated with radiotherapy alone, as long as it is localized.[23] Advanced Hodgkin disease requires systemic chemotherapy, sometimes combined with radiotherapy.[24]Chemotherapy used includes the ABVDregimen.

EditPrognosis

5-year relative survival by stage at diagnosis[25]
Stage at diagnosis 5-year relative
survival (%)
Percentage
of cases (%)
Localized (confined to primary site) 82.1 28
Regional (spread to regional lymph nodes) 77.5 19
Distant (cancer has metastasized) 59.9 45
Unknown (unstaged) 67.5 8

Epidemiology

History

Research directions

References

External links

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