出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/06/26 12:58:23」(JST)
Non-Hodgkin's Lymphomas (NHL) | |
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Classification and external resources | |
Micrograph of mantle cell lymphoma, a type of non-Hodgkin lymphoma. Terminal ileum. H&E stain. |
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ICD-10 | C82-C85 |
ICD-9 | 200, 202 |
ICD-O: | 9591/3 |
OMIM | 605027 |
DiseasesDB | 9065 |
MedlinePlus | 000581 |
eMedicine | med/1363 ped/1343 |
MeSH | D008228 |
The non-Hodgkin lymphomas (NHLs) are diverse group of blood cancers that include any kind of lymphoma except Hodgkin's lymphomas.[1] Types of NHL vary significantly in their severity, from indolent to very aggressive.
Lymphomas are types of cancer derived from lymphocytes, a type of white blood cell. Lymphomas are treated by combinations of chemotherapy, monoclonal antibodies, immunotherapy, radiation, and hematopoietic stem cell transplantation.
Non-Hodgkin lymphomas were classified according to the 1982 Working Formulation which recognizes 16 types. The Working Formulation is now considered obsolete, and the classification is commonly used primarily for statistical comparisons with previous decades. The Working Formulation has been superseded twice.
The latest lymphoma classification, the 2008 WHO classification, largely abandoned the "Hodgkin" vs. "Non-Hodgkin" grouping. Instead, it lists over 80 different forms of lymphomas in four broad groups.[2]
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Hodgkin lymphoma (HL, Hodgkin disease), described by Thomas Hodgkin in 1832, was the first form of lymphoma described and defined. Other forms were later described and there was a need to classify them. Because Hodgkin lymphoma was much more radiation-sensitive than other forms, its diagnosis was important for oncologists and their patients. Thus, research originally focused on it. The first classification of Hodgkin lymphoma was proposed by Robert J. Luke in 1963.
While consensus was rapidly reached on the classification of Hodgkin lymphoma, there remained a large group of very different diseases requiring further classification. The Rappaport classification, proposed by Henry Rappaport in 1956 and 1966, became the first widely accepted classification of lymphomas other than Hodgkin. Following its publication in 1982, the Working Formulation became the standard classification for this group of diseases. It introduced the term non-Hodgkin lymphoma (NHL) and defined three grades of lymphoma.
However, NHL consists of 16 different conditions that have little in common with each other. They are grouped by their aggressiveness. Less aggressive non-Hodgkin lymphomas are compatible with a long survival while more aggressive non-Hodgkin lymphomas can be rapidly fatal without treatment. Without further narrowing, the label is of limited usefulness for patients or doctors.
Nevertheless, the Working Formulation and the NHL category continue to be used by many. To this day, lymphoma statistics are compiled as Hodgkin's vs non-Hodgkin lymphomas by major cancer agencies, including the National Cancer Institute in its SEER program, the Canadian Cancer Society and the IARC.
The Centre for Disease Control and Prevention (CDC) included certain types of non-Hodgkin's lymphoma as AIDS-defining cancers in 1987.[3] Immune suppression rather than HIV itself is implicated in the pathogenesis of this malignancy, with a clear correlation between the degree of immune suppression and the risk of developing NHL.[4] HIV-infected patients are at an increased risk for developing both Hodgkin lymphoma and NHL when compared with the general population.
The many different forms of lymphoma likely have different causes. These possible causes and associations with at least some forms of NHL include:
Some studies have shown a causal link between non-Hodgkin lymphoma and exposure to polychlorinated biphenyls (PCBs), a persistent organic pollutant now found throughout the natural environment.[6][7] PCBs were widely used as dielectric and coolant fluids in transformers, capacitors, and electric motors. Due to the toxicity of PCBs and their classification as persistent organic pollutants, PCB production was banned by the U.S. Congress in 1977 and by the Stockholm Convention on Persistent Organic Pollutants in 2001.[8][9] Though they are no longer produced, PCBs do not readily break down once they have been released into the environment.
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年齢 | 子供における頻度 | 男性(%) | ステージI,II vs III,IV(%) | B症状(%) | 骨髄浸潤(%) | 消化管浸潤(%) | 5年生存率(%) | |
B細胞CLL/小リンパ球性リンパ腫 | 65 | まれ | 53 | 9 vs 91 | 33 | 72 | 3 | 51 |
マントル細胞リンパ腫 | 63 | まれ | 74 | 20 vs 80 | 28 | 64 | 9 | 27 |
MALT型の節外辺縁域B細胞リンパ腫 | 60 | まれ | 48 | 67 vs 33 | 19 | 14 | 50 | 74 |
濾胞リンパ腫 | 59 | まれ | 42 | 33 vs 67 | 28 | 42 | 4 | 72 |
びまん性大細胞型B細胞リンパ腫 | 64 | ~25% | 55 | 54 vs 46 | 33 | 16 | 18 | 46 |
バーキットリンパ腫 | 31 | ~30% | 89 | 62 vs 38 | 22 | 33 | 11 | 45 |
前駆T細胞リンパ芽球性リンパ腫 | 28 | ~40% | 64 | 11 vs 89 | 21 | 50 | 4 | 26 |
未分化大細胞型リンパ腫 | 34 | よくある | 69 | 51 vs 49 | 53 | 13 | 9 | 77 |
末梢型T細胞非ホジキンリンパ腫 | 61 | ~5% | 55 | 20 vs 80 | 50 | 36 | 15 | 25 |
進行スピードによる分類 | 該当する非ホジキンリンパ腫の種類 |
低悪性度(年単位で進行) | 濾胞性リンパ腫 MALTリンパ腫 |
中悪性度(月単位で進行) | びまん性大細胞性B細胞性リンパ腫 未分化大細胞リンパ腫 |
高悪性度(週単位で進行) | リンパ芽球性リンパ腫 バーキットリンパ腫 |
小型非切れ込み核細胞性リンパ腫、小型非きれこみ核細胞性リンパ腫、小型非分割細胞リンパ腫
小切れ込み核細胞型びまん性リンパ腫、びまん性小分割細胞性リンパ腫
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