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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
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| {{DiseaseDisorder infobox |
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| Name = Hodgkin's lymphoma |
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| ICD10 = {{ICD10|C|81||c|81}} |
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| ICD9 = {{ICD9|201}} |
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| ICDO = 9650/3-9667/3 |
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| Image = Hodgkin_lymphoma_(1)_mixed_cellulary_type.jpg|
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| Width = 200|
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| Caption = Lymph node biopsy showing Hodgkin's lymphoma.|
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| OMIM = |
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| MedlinePlus = 000580 |
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| DiseasesDB = 5973 |
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| }}
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| {{SI}}
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| {{CMG}}
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| __NOTOC__ | | __NOTOC__ |
| {{Editor Help}}
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| ==Overview==
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| '''Hodgkin's lymphoma''', also known as '''Hodgkin's disease''', is a type of [[lymphoma]] first described by [[Thomas Hodgkin]] in 1832. Hodgkin's lymphoma is characterized clinically by the orderly spread of disease from one [[lymph node]] group to another and by the development of [[B symptoms|systemic symptoms]] with advanced disease. Pathologically, the disease is characterized by the presence of [[Reed-Sternberg cell]]s. Hodgkin's lymphoma was one of the first [[cancer]]s to be cured by radiation. Later it was one of the first to be cured by [[history of cancer chemotherapy#combination chemotherapy|combination chemotherapy]]. The cure rate is about 93%, making it one of the most curable forms of cancer.
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| == Epidemiology ==
| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''{{Hodgkin's lymphoma}} |
| Unlike some [[non-Hodgkin lymphoma|other lymphomas]], whose [[incidence (epidemiology)|incidence]] increases with age, Hodgkin's lymphoma has a bimodal incidence curve; that is, it occurs most frequently in two separate age groups, the first being young adulthood (age 15–35) and the second being in those over 55 years old although these peaks may vary slightly with nationality.<ref> {{cite book | last=Mauch | first=Peter | coauthors=James Armitage, Volker Diehl, Richard Hoppe, Laurence Weiss | title=Hodgkin's Disease | publisher=[[Lippincott Williams & Wilkins]] | date=1999 | pages=62-64 | isbn=0-7817-1502-4}} </ref> Overall, it is more common in men, except for the ''[[nodular sclerosis]]'' variant (see below), which is more common in women.
| | {{CMG}}; {{AE}} {{AS}} {{M.B}}; {{GRR}} {{NWS}} |
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| The annual incidence of Hodgkin's lymphoma is about 1/25,000 people, and the disease accounts for slightly less than 1% of all cancers worldwide.
| | {{SK}} Hodgkin's disease; HL; Nodular lymphocyte predominance Hodgkin's lymphoma; Classical Hodgkin's lymphoma; CHL; Nodular sclerosing Hodgkin's lymphoma; Mixed cellularity Hodgkin's lymphoma; Lymphocyte rich Hodgkin's lymphoma; Lymphocyte depleted Hodgkin's lymphoma. |
| | ==[[Hodgkin's lymphoma overview|Overview]]== |
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| The incidence of Hodgkin's lymphoma is increased in patients with HIV infection.<ref>{{cite journal |author=Biggar RJ, Jaffe ES, Goedert JJ, Chaturvedi A, Pfeiffer R, Engels EA |title=Hodgkin lymphoma and immunodeficiency in persons with HIV/AIDS |journal=Blood |volume=108 |issue=12 |pages=3786-91 |year=2006 |pmid=16917006 |doi=10.1182/blood-2006-05-024109}}</ref> In contrast to many other lymphomas associated with HIV infection it occurs most commonly in patients with higher CD4 T cell counts.
| | ==[[Hodgkin's lymphoma historical perspective|Historical Perspective]]== |
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| == Symptoms == | | ==[[Hodgkin's lymphoma classification|Classification]]== |
| Swollen but painless [[lymph node]]s are the most common sign of Hodgkin's lymphoma, often occurring in the neck. The lymph nodes of the chest are often affected and these may be noticed on a chest x-ray.
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| Splenomegaly, or enlargement of the spleen, occurs in about 30% of people with Hodgkin's lymphoma. The enlargement, however, is seldom massive. The liver may also be enlarged due to liver involvement in the disease in about 5% of cases.
| | ==[[Hodgkin's lymphoma pathophysiology|Pathophysiology]]== |
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| About one-third of people with Hodgkin's disease may also notice some systemic symptoms, such as low-grade fever, night sweats, weight loss, itchy skin ([[itch|pruritus]]), or [[fatigue (physical)|fatigue]]. Classically, involved nodes are painful after alcohol consumption, though this phenomenon is rare. Patients may also present with a cyclic high-grade fever known as [[Pel-Ebstein fever]], although there is debate as to whether or not this truly exists. <ref> {{cite journal | last=Asher | first=Richard | title=Making Sense | journal=The New England Journal of Medicine | volume=333 | pages 66-67 | date=[[July 6]], [[1995]] }} </ref> Systemic symptoms such as fever and weight loss are known as [[B symptoms]].
| | ==[[Hodgkin's lymphoma causes|Causes]]== |
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| == Diagnosis == | | ==[[Hodgkin's lymphoma differential diagnosis|Differentiating Hodgkin's lymphoma from other Diseases]]== |
| Hodgkin's lymphoma must be distinguished from non-cancerous causes of lymph node swelling (such as various infections) and from other types of cancer. Definitive diagnosis is by lymph node [[biopsy]] (removal of a piece of lymph node tissue for pathological examination). [[Blood test]]s are also performed to assess function of major organs and to assess safety for [[chemotherapy]]. [[Positron emission tomography]] (PET) is used to detect small deposits that do not show on CT scanning. In some cases a [[Gallium imaging|Gallium Scan]] may be used instead of a PET scan. | |
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| ===Pathology=== | | ==[[Hodgkin's lymphoma epidemiology and demographics|Epidemiology and Demographics]]== |
| [[Image:Reed-Sternberg (Hodgkin's Lymphoma).jpg|thumb|left|Reed-Sternberg Hodgkin's Lymphoma<ref>http://picasaweb.google.com/mcmumbi/USMLEIIImages</ref>]] | |
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| ====Macroscopy==== | | ==[[Hodgkin's lymphoma risk factors|Risk Factors]]== |
| Affected lymph nodes (most often, laterocervical lymph nodes) are enlarged, but their shape is preserved because the capsule is not invaded. Usually, the cut surface is white-grey and uniform; in some histological subtypes (e.g. [[nodular sclerosis]]) may appear a nodular aspect
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| ====Microscopy==== | | ==[[Hodgkin's lymphoma screening|Screening]]== |
| Microscopic examination of the lymph node biopsy reveals complete or partial effacement of the lymph node architecture by scattered large malignant cells known as Reed-Sternberg cells (typical and variants) admixed within a reactive cell infiltrate composed of variable proportions of lymphocytes, histiocytes, eosinophils, and plasma cells. The Reed-Sternberg cells are identified as large often bi-nucleated cells with prominent nucleoli and an unusual [[CD45]]-, [[CD30]]+, [[CD15]]+/- immunophenotype. In approximately 50% of cases, the Reed-Sternberg cells are infected by the [[Epstein-Barr]] [[virus]].
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| Characteristics of classic Reed-Sternberg cells include large size (20–50 micrometres), abundant, amphophilic, finely granular/homogeneous cytoplasm; two mirror-image nuclei (owl eyes) each with an eosinophilic nucleolus and a thick nuclear membrane ([[chromatin]] is distributed at the cell periphery).
| | ==[[Hodgkin's lymphoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| Variants:
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| * Hodgkin's cell (atypical mononuclear RSC) is a variant of RS cell, which has the same characteristics, but is mononucleated.
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| * Lacunar RSC is large, with a single hyperlobated nucleus, multiple, small nucleoli and eosinophilic cytoplasm which is retracted around the nucleus, creating an empty space ("lacunae").
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| * Pleomorphic RSC has multiple irregular nuclei.
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| * "Popcorn" RSC (lympho-histiocytic variant) is a small cell, with a very lobulated nucleus, small nucleoli.
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| * "Mummy" RSC has a compact nucleus, no nucleolus and basophilic cytoplasm. <ref>[http://www.pathologyatlas.ro/Reed%20Sternberg%20Cell%20Hodgkin%20Lymphoma.html]</ref>
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| Hodgkin's lymphoma can be sub-classified by histological type. The cell histology in Hodgkin's lymphoma is not as important as it is in [[non-Hodgkin's lymphoma]]: the treatment and prognosis in Hodgkin's lymphoma depend on the stage of disease rather than the histotype. | | [[Hodgkin's lymphoma diagnostic study of choice|Diagnostic Study of Choice]] | [[Hodgkin's lymphoma history and symptoms|History and Symptoms]] | [[Hodgkin's lymphoma physical examination|Physical Examination]] | [[Hodgkin's lymphoma laboratory tests|Laboratory Findings]] | [[Hodgkin's lymphoma x ray findings|X Ray Findings]] | [[Hodgkin's lymphoma CT|CT]] | [[Hodgkin's lymphoma MRI|MRI]] | [[Hodgkin's lymphoma ultrasound|Ultrasound]] | [[Hodgkin's lymphoma biopsy|Biopsy]] | [[Hodgkin's lymphoma other imaging findings|Other Imaging Findings]] | [[Hodgkin's lymphoma other diagnostic studies|Other Diagnostic Studies]] |
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| == Types == | | ==Treatment== |
| ===Classical ===
| | [[Hodgkin's lymphoma medical therapy|Medical Therapy]] | [[Hodgkin's lymphoma surgery|Surgery]] | [[Hodgkin's lymphoma primary prevention|Primary Prevention]] | [[Hodgkin's lymphoma secondary prevention|Secondary Prevention]] | [[Hodgkin's lymphoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Hodgkin's lymphoma future or investigational therapies|Future or Investigational Therapies]] |
| Classical Hodgkin's lymphoma (excluding nodular lymphocyte predominant Hodgkin's) can be subclassified into 4 pathologic subtypes based upon Reed-Sternberg cell morphology and the composition of the reactive cell infiltrate seen in the lymph node biopsy specimen. ({{ICDO|9650|3}})
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| Classic Hodgkin's Lymphoma (CHL) subtypes:
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| # Nodular sclerosing CHL is the most common subtype and is composed of large [[tumor]] nodules with lacunar RS cells subtype composed of numerous classic often pleomorphic RS cells with only few reactive lymphocytes which may easily be confused with diffuse large cell lymphoma. ({{ICD10|C|81|3|c|81}}, {{ICDO|9653|3}})
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| # Mixed-cellularity subtype is a common subtype and is composed of numerous classic RS cells admixed with numerous inflammatory cells including lymphocytes, histiocytes, eosinophils, and plasma cells. ({{ICD10|C|81|2|c|81}}, {{ICDO|9652|3}})
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| # Lymphocyte-rich and
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| # lymphocyte depleted are rare subtypes.
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| === Other === | | ==Related Chapters== |
| Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) is no longer classified as a form of classic Hodgkin's lymphoma. This is due to the fact that the RSC variants (popcorn cells) that characterize this form of the disease invariably express B lymphocyte markers such as CD20 (thus making NLPHL an unusual form of B cell lymphoma), and that (unlike classic HL) NLPHL may progress to [[B-cell lymphoma|diffuse large B cell lymphoma]]. There are small but clear differences in prognosis between the various forms.
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| Lymphocyte predominant HL is an uncommon subtype composed of vague nodules of numerous reactive lymphocytes admixed with large popcorn-shaped RSC. Unlike classic RSC, the non-classic popcorn-shaped RS cells of NLPHL are CD15 and CD30 negative while positive for the B cell marker CD20. ({{ICD10|C|81|0|c|81}}, {{ICDO|9659|3}})
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| == Staging ==
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| After Hodgkin's lymphoma is diagnosed, a patient will be ''[[Cancer staging|staged]]'': that is, they will undergo a series of tests and procedures which will determine what areas of the body are affected. These procedures will include documentation of their histology, a physical examination, blood tests, chest [[X-ray]] radiographs, [[computed tomography]] (CT) scans or [[magnetic resonance imaging]] (MRI) scans of the chest, abdomen and pelvis, and a bone marrow biopsy. [[Positron emission tomography]] (PET) scan is now used instead of the [[gallium scan]] for staging. In the past, a [[lymphangiogram]] or surgical [[laparotomy]] (which involves opening the abdominal cavity and visually inspecting for tumors) were performed. Lymphangiograms or laparotomies are very rarely performed, having been supplanted by improvements in imaging with the CT scan and PET scan.
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| On the basis of this staging, the patient will be classified according to a staging classification (the Ann Arbor staging classification scheme is a common one):
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| * Stage I is involvement of a single lymph node region (I) or single extralymphatic site (Ie);
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| * Stage II is involvement of two or more lymph node regions on the same side of the [[diaphragm (anatomy)|diaphragm]] (II) or of one lymph node region and a contiguous extralymphatic site (IIe);
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| * Stage III is involvement of lymph node regions on both sides of the diaphragm, which may include the [[spleen]] (IIIs) and/or limited contiguous extralymphatic organ or site (IIIe, IIIes);
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| * Stage IV is disseminated involvement of one or more extralymphatic organs.
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| The absence of systemic symptoms is signified by adding 'A' to the stage; the presence of systemic symptoms is signified by adding 'B' to the stage.
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| ==Prognosis==
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| In 1998, an international effort<ref> {{cite journal | last=Hasenclever | coauthour=Diehl, et. al | title=A Prognostic Score for Advanced Hodgkin's Disease | journal=New England Journal of Medicine | volume=339 | issue=21 | date=[[1998-11-19]]}} </ref> identified seven prognostic factors that accurately predict the success rate of conventional treatment in patients with locally extensive or advanced stage Hodgkin's lymphoma. Freedom from progression (FFP) at 5 years was directly related to the number of factors present in a patient. The 5-year FFP for patients with zero factors is 84%. Each additional factor lowers the 5-year FFP rate by 7%, such that the 5-year FFP for a patient with 5 or more factors is 42%.
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| The adverse prognostic factors identified in the international study are:
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| * Age >= 45 years
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| * Stage IV disease
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| * Hemoglobin < 10.5 g/dl
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| * Lymphocyte count < 600/µl or < 8%
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| * Male
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| * Albumin < 4.0 g/dl
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| * White blood count >= 15,000/µl
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| Other studies have reported the following to be the most important adverse prognostic factors: mixed-cellularity or lymphocyte-depleted histologies, male sex, large number of involved nodal sites, advanced stage, age of 40 years or more, the presence of B symptoms, high [[erythrocyte sedimentation rate]], and bulky disease (widening of the [[mediastinum]] by more than one third, or the presence of a nodal mass measuring more than 10 cm in any dimension.)
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| == Treatment ==
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| {{see also | ABVD}}
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| Patients with early stage disease (IA or IIA) are effectively treated with [[radiation]] therapy or chemotherapy. The choice of treatment depends on the age, sex, bulk and the histological subtype of the disease. Patients with later disease (III, IVA, or IVB) are treated with combination chemotherapy alone. Patients of any stage with a large mass in the chest are usually treated with combined chemotherapy and radiation therapy.
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| Currently, the ''[[ABVD]]'' [[chemotherapy]] regimen is the gold standard for treatment of Hodgkin's disease. The abbreviation stands for the four drugs [[Adriamycin]], [[bleomycin]], [[vinblastine]], and [[dacarbazine]]. Developed in Italy in the 1970s, the ABVD treatment typically takes between six and eight months, although longer treatments may be required. Another form of treatment is the newer [[Stanford V]] regimen, which is typically only half as long as the ABVD but which involves a more intensive chemotherapy schedule and incorporates radiation therapy. However, in a randomized controlled study, Stanford V was inferior.<ref name="pmid16172458">{{cite journal |author=Gobbi PG, Levis A, Chisesi T, ''et al'' |title=ABVD versus modified stanford V versus MOPPEBVCAD with optional and limited radiotherapy in intermediate- and advanced-stage Hodgkin's lymphoma: final results of a multicenter randomized trial by the Intergruppo Italiano Linfomi |journal=J. Clin. Oncol. |volume=23 |issue=36 |pages=9198-207 |year=2005 |pmid=16172458 |doi=10.1200/JCO.2005.02.907}}</ref>
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| Another form of treatment, mainly in Europe for stages > II is [[BEACOPP]]. The cure rate with the BEACOPP esc. regimen is approximately 10-15% higher than with standard ABVD in advanced stages. Although this was shown in a landmark paper in The New England Journal of Medicine (Diehl et al.), the US physicians still favor ABVD. Probably because some physicians think that BEACOPP induces more secondary leukemia. However, this seems negligible compared to the higher cure rates. Also, BEACOPP is more expensive because of the G-CSF-support that is required. Currently, the German Hodgkin Study group tests 8x BEACOPP esc vs. 6x BEACOPP esc vs. 8x BEACOPP-14 baseline (HD15-trial).
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| With appropriate treatment, over 93% of Hodgkin's lymphoma cases are curable.
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| The high cure rates and long survival of many patients with Hodgkin's lymphoma has led to a high concern with late adverse effects of treatment, including cardiovascular disease and second malignancies such as acute [[leukemia]]s, lymphomas, and solid tumors within the radiation therapy field. Most patients with early stage disease are now treated with abbreviated chemotherapy and involved-field radiation therapy rather than with radiation therapy alone. Clinical research strategies are exploring reduction of the duration of chemotherapy and dose and volume of radiation therapy in an attempt to reduce late morbidity and mortality of treatment while maintaining high cure rates. Hospitals are also treating those who respond quickly to chemo-therapy with no radiation.
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| ==See also==
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| * [[ABVD]] | | * [[ABVD]] |
| * [[Stanford V]] | | * [[Stanford V]] |
| * [[Non-Hodgkin lymphoma]] | | * [[Non-Hodgkin lymphoma]] |
| | * [[The Heart in Hodgkin Lymphoma]] |
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| ==References== | | ==Resources== |
| {{reflist|2}}
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| ==External links==
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| * [http://www.leukaemiacare.org.uk Leukaemia CARE] | | * [http://www.leukaemiacare.org.uk Leukaemia CARE] |
| * [http://www.cancer.gov/cancertopics/types/hodgkinslymphoma Hodgkin's Lymphoma Home Page] at the American [[National Cancer Institute]] | | * [http://www.cancer.gov/cancertopics/types/hodgkinslymphoma Hodgkin's Lymphoma Home Page] at the American [[National Cancer Institute]] |
| * [http://www.lymphoma.org.uk/ Lymphoma Association] | | * [http://www.lymphoma.org.uk/ Lymphoma Association] |
| * [http://www.ahln.org/ Australian Hodgkin's Lymphoma Network] | | * [http://www.ahln.org/ Australian Hodgkin's Lymphoma Network] |
| * [http://www.lymphomainfo.net/hodgkins/timeline.html Timeline of discovery and treatment of Hodgkin's Lymphoma]
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| * [http://www.kids-cancer.org/lymphomas.htm Lymphomas in Children and Adolescents] The Centre for Cancer and Blood Disorders at Sydney Children’s Hospital provides information on cancers in children and adolescents, including Hodgkin and Non-Hodgkins Lymphomas. | | * [http://www.kids-cancer.org/lymphomas.htm Lymphomas in Children and Adolescents] The Centre for Cancer and Blood Disorders at Sydney Children’s Hospital provides information on cancers in children and adolescents, including Hodgkin and Non-Hodgkins Lymphomas. |
| * [http://www.lymphomainfo.net/hodgkins/description.html Hodgkin's Lymphoma]
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| * [http://www.merck.com/mmpe/sec11/ch143/ch143b.html Hodgkin Disease from The Merck Manual]
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| * [http://www.pathologyatlas.ro/Reed%20Sternberg%20Cell%20Hodgkin%20Lymphoma.html Photos at Atlas of Pathology]
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| * [http://cancer.stanford.edu/trials/adult/Hodgkins_Disease/ Hodgkin's trials at Stanford University]
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| * [http://www.leukemia-lymphoma.org/all_page?item_id=7085 The Leukemia & Lymphoma Society] | | * [http://www.leukemia-lymphoma.org/all_page?item_id=7085 The Leukemia & Lymphoma Society] |
| * [http://www.alesecoco.org/ Journal Of A 6 Year Battle with Hodgkins written by Alese Coco (March 10 1984 - May 7 2007)]. | | * [http://www.alesecoco.org/ Journal Of A 6 Year Battle with Hodgkins written by Alese Coco (March 10 1984 - May 7 2007)]. |
| * [http://www.antibodypatterns.com/tr.php Anti-Tr antibody]
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| * [http://forums.webmagic.com/ubbthreads/postlist.php?Cat=&Board=UBB1/ Message board for those who have Hodgkin's or in remission]
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| * [http://www.lymphoma.ie Lymphoma Support Ireland - Information & Patient Stories Online] | | * [http://www.lymphoma.ie Lymphoma Support Ireland - Information & Patient Stories Online] |
| * [http://www.hodgkinsdisease.org/ The Hodgkin's Disease List] A support group for those afflicted with Hodgkin's Disease
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