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Hodgkin's lymphoma
Classification and external resources
Lymph node biopsy showing Hodgkin's lymphoma.
ICD-10 C81.
ICD-9 201
ICD-O: 9650/3-9667/3
DiseasesDB 5973
MedlinePlus 000580
eMedicine med/1022 
MeSH D006689

Hodgkin's lymphoma, also known as Hodgkin's disease, is a type of lymphoma first described by Thomas Hodgkin in 1832. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. MedlinePlus, with the MedlinePlus Medical Encyclopedia, is a website network containing Health information from the world's largest medical Library eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books Lymphoma a type of Neoplasm that originates in Lymphocytes (a type of White blood cell in the vertebrate Immune system) For the British historian his nephew with the same name see Thomas Hodgkin. Hodgkin's lymphoma is characterized clinically by the orderly spread of disease from one lymph node group to another and by the development of systemic symptoms with advanced disease. A Lymph node ( lɪmf noʊd is an organ consisting of many types of cells and is a part of the Lymphatic system. B symptoms refer to Systemic symptoms of Fever, Night sweats and Weight loss which can be associated with both Hodgkin's lymphoma Pathologically, the disease is characterized by the presence of Reed-Sternberg cells. Reed-Sternberg cells (Lacunar Histiocytes for certain types are different giant cells found on Light microscopy in biopsies from individuals with Hodgkin's Hodgkin's lymphoma was one of the first cancers to be cured by radiation. Cancer (medical term Malignant Neoplasm) is a class of Diseases in which a group of cells display uncontrolled Later it was one of the first to be cured by combination chemotherapy. The era of cancer Chemotherapy began in the 1940s with the first use of Nitrogen mustards and Folic acid antagonist drugs The cure rate is about 93%, making it one of the most curable forms of cancer—if it is detected in the early stages. Later-stage cancers show a significantly worse prognosis[1]. The Handbook of Cancer Chemotherapy, Sixth Edition states that "the potential for cure should not lead clinicians and patients to lose sight of the fact that [Hodgkin's lymphoma] is a malignancy and that approximately 20% to 25% of patients. . . die of the disease. "[2]

Contents

Epidemiology

Unlike some other lymphomas, whose 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. The non-Hodgkin lymphomas are a diverse group of hematologic cancers which encompass any Lymphoma other than Hodgkin lymphoma. Incidence is a measure of the risk of developing some new condition within a specified period of time [3] Overall, it is more common in males, except for the nodular sclerosis variant (see below), which is more common in females. Nodular sclerosis (or "NSHL" is a form of Hodgkin's lymphoma which is the most common subtype of HL in developed countries

The annual incidence of Hodgkin's lymphoma is about one in 25,000 people, and the disease accounts for slightly less than 1% of all cancers worldwide.

The incidence of Hodgkin's lymphoma is increased in patients with HIV infection. [4] In contrast to many other lymphomas associated with HIV infection it occurs most commonly in patients with higher CD4 T cell counts.

Symptoms

Patients with Hodgkin's lymphoma may present with the following symptoms:

Diagnosis

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 (Usually excisional biopsy with microscopic examination). A biopsy (in Greek: βίος life and όψη look/appearance is a Medical test involving the removal of cells or tissues Blood tests are also performed to assess function of major organs and to assess safety for chemotherapy. A blood test is a laboratory analysis performed on a Blood sample that is usually extracted from a Vein in the arm using a needle, or via Chemotherapy, in its most general sense refers to treatment of disease by chemicals that kill cells specifically those of micro-organisms or Cancer. Positron emission tomography (PET) is used to detect small deposits that do not show on CT scanning. Positron emission tomography ( PET) is a Nuclear medicine imaging technique which produces a three-dimensional image or map of functional processes in the In some cases a Gallium Scan may be used instead of a PET scan. Gallium imaging is a Nuclear medicine method for the detection of Infections and Cancers Radioactive Gallium -67 binds to

Pathology

Macroscopy

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) a nodular aspect may appear. Nodular sclerosis (or "NSHL" is a form of Hodgkin's lymphoma which is the most common subtype of HL in developed countries

Microscopy

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 Immunology, the CD45 Antigen (CD stands for Cluster of differentiation) is a Protein which was originally called leukocyte common antigen CD30, also known as TNFRSF8, is a cell membrane CD15 (3-fucosyl-N-acetyl-lactosamine is a Cluster of differentiation - an immunologically significant molecule In approximately 50% of cases, the Reed-Sternberg cells are infected by the Epstein-Barr virus. The Epstein-Barr Virus ( EBV) also called Human herpesvirus 4 (HHV-4 is a Virus of the herpes family (which includes Herpes A virus (from the Latin virus meaning Toxin or Poison) is a sub-microscopic infectious agent that is unable

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). Chromatin is the complex basis of DNA and protein that makes up Chromosomes It is found inside the nuclei of eukaryotic cells, and within the

Variants:

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. The non-Hodgkin lymphomas are a diverse group of hematologic cancers which encompass any Lymphoma other than Hodgkin lymphoma.

Types

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. (M9650/3)

Family Name Description ICD-10 ICD-O
Classical 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. The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems See also Cancer A tumor or tumour is the name for a swelling or lesion formed by an abnormal growth of cells (termed neoplastic C81.1 M9663/3
Classical 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. The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems C81.2 M9652/3
Classical Lymphocyte-rich Is a rare subtype. The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems A lymphocyte is a type of White blood cell in the Vertebrate Immune system. C81.0 M9651/3
Classical Lymphocyte depleted Is a rare subtype. The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems C81.3 M9653/3
Non-classical Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) Is no longer classified as a form of classic Hodgkin's lymphoma. The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems 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 diffuse large B cell lymphoma. The B-cell lymphomas are types of Non-Hodgkin lymphoma affecting B cells. There are small but clear differences in prognosis between the various forms. 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. CD15 (3-fucosyl-N-acetyl-lactosamine is a Cluster of differentiation - an immunologically significant molecule CD30, also known as TNFRSF8, is a cell membrane CD20 is a non- Glycosylated phosphoprotein expressed on the surface of all mature B-cells. C81.7 M9659/3

Staging

After Hodgkin's lymphoma is diagnosed, a patient will be staged: that is, they will undergo a series of tests and procedures which will determine what areas of the body are affected. The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems The stage of a cancer is a descriptor (usually numbers I to IV of how much the Cancer has spread 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. X-radiation (composed of X-rays) is a form of Electromagnetic radiation. Computed tomography (CT is a Medical imaging method employing Tomography. Positron emission tomography (PET) scan is now used instead of the gallium scan for staging. Positron emission tomography ( PET) is a Nuclear medicine imaging technique which produces a three-dimensional image or map of functional processes in the A gallium scan or gallium 67 scan is a type of Nuclear medicine study that uses a radioactive tracer to obtain images of a specific type of tissue or disease In the past, a lymphangiogram or surgical laparotomy (which involves opening the abdominal cavity and visually inspecting for tumors) were performed. A lymphangiogram is a medical test whereby pictures of the Lymphatic system taken with X-rays after a special dye is injected to outline the Lymph nodes A laparotomy is a surgical procedure involving an incision through the Abdominal wall to gain access into the Abdominal cavity. Lymphangiograms or laparotomies are very rarely performed, having been supplanted by improvements in imaging with the CT scan and PET scan

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):

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.

Prognosis

In 1998, an international effort[11] 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%.

The adverse prognostic factors identified in the international study are:

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. The erythrocyte sedimentation rate (ESR also called a sedimentation rate, sed rate, or Biernacki Reaction, is the rate at which Red blood cells The mediastinum is a non-delineated group of structures in the Thorax (chest surrounded by Loose connective tissue. )

Treatment

See also: ABVD

Patients with early stage disease (IA or IIA) are effectively treated with radiation therapy or chemotherapy. ABVD is a Chemotherapy regimen used in the first-line treatment of Hodgkin lymphoma. Radiation therapy (or radiotherapy) is the medical use of Ionizing radiation as part of Cancer treatment to control Malignant 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.

Currently, the ABVD chemotherapy regimen is the gold standard for treatment of Hodgkin's disease. ABVD is a Chemotherapy regimen used in the first-line treatment of Hodgkin lymphoma. Chemotherapy regimens are often identified with acronyms identifying the agents used in combination The abbreviation stands for the four drugs Adriamycin, bleomycin, vinblastine, and dacarbazine. Doxorubicin ( INN,; trade name Adriamycin; also known as hydroxydaunorubicin) is a drug used in cancer Chemotherapy. Bleomycin is a Glycopeptide antibiotic produced by the Bacterium Streptomyces verticillus. Vinblastine is an anti-mitotic drug used to treat certain kinds of Cancer, including Hodgkin's lymphoma, non-small cell Lung cancer Dacarbazine (da-KAR-ba-zeen (brand names DTIC DTIC-Dome also known as DIC or Imidazole Carboxamide is an antineoplastic Chemotherapy drug used in the treatment of various 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. Stanford V is a Chemotherapy regimen designed for Hodgkin's lymphoma that consists of the following Mechlorethamine (Sometimes Cyclophosphamide However, in a randomized controlled study, Stanford V was inferior. [12]

Another form of treatment, mainly in Europe for stages > II is BEACOPP. BEACOPP is a Chemotherapy regimen for treatment of Hodgkin's lymphoma in Stages > II 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).

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 leukemias, lymphomas, and solid tumors within the radiation therapy field. Leukemia or leukaemia (Greek leukos λευκός, "white" aima αίμα, "blood" is a Cancer of the Blood 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.

Notable cases

Cultural references

See also

References

  1. ^ Stein, RS. Donald Joseph “DJ” Qualls (born June 12, 1978) is an American Fashion model and Actor. ABVD is a Chemotherapy regimen used in the first-line treatment of Hodgkin lymphoma. Stanford V is a Chemotherapy regimen designed for Hodgkin's lymphoma that consists of the following Mechlorethamine (Sometimes Cyclophosphamide The non-Hodgkin lymphomas are a diverse group of hematologic cancers which encompass any Lymphoma other than Hodgkin lymphoma. ; Morgan, D (2003). Handbook of Cancer Chemotherapy, Sixth Edition. Lippincott Williams & Wilkins, 493, Table 21. Lippincott Williams & Wilkins is an academic and professional medical publisher founded in 1792 and now a part of the Wolters Kluwer group 2: "Hodgkin's Disease: Incidence of stages and results of therapy. " ISBN 0-7817-3629-3.
  2. ^ Ibid. , 489. ISBN 0-7817-3629-3.
  3. ^ Mauch, Peter; James Armitage, Volker Diehl, Richard Hoppe, Laurence Weiss (1999). Hodgkin's Disease. Lippincott Williams & Wilkins, 62-64. Lippincott Williams & Wilkins is an academic and professional medical publisher founded in 1792 and now a part of the Wolters Kluwer group ISBN 0-7817-1502-4.  
  4. ^ Biggar RJ, Jaffe ES, Goedert JJ, Chaturvedi A, Pfeiffer R, Engels EA (2006). "Hodgkin lymphoma and immunodeficiency in persons with HIV/AIDS". Blood 108 (12): 3786-91. doi:10.1182/blood-2006-05-024109. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 16917006.  
  5. ^ Howard Terebelo, DO, FACP: discussion with patient, July 5, 2007.
  6. ^ [Anonymous]. Symptoms & Signs. The Lymphomas. 2006: 11.
  7. ^ Ibid. , 13,14.
  8. ^ RK Gupta, MB, PhD, FRCP; MK Gospodarowicz, MD, FRCPC; T. Andrew Lister, MD. Clinical Evaluation and Staging of Hodgkin's Disease. Hodgkin's Disease: 1999; 230-1. ISBN 0-7187-150204
  9. ^ Asher, Richard (July 6, 1995). Events 1044 - The Battle of Ménfő takes place 1189 - Richard the Lionheart is crowned King of England Year 1995 ( MCMXCV) was a Common year starting on Sunday. Events of 1995 "Making Sense". The New England Journal of Medicine 333.  
  10. ^ Reed-Sternberg cell - Hodgkin lymphoma
  11. ^ Hasenclever D, Diehl V (1998-11-19). Year 1998 ( MCMXCVIII) was a Common year starting on Thursday (link will display full 1998 Gregorian calendar) Events 1095 - The Council of Clermont, called by Pope Urban II to discuss sending the First Crusade to the Holy Land "A Prognostic Score for Advanced Hodgkin's Disease". New England Journal of Medicine 339 (21): 1506-14. doi:10.1056/NEJM199811193392104. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 9819449.  
  12. ^ Gobbi PG, Levis A, Chisesi T, et al (2005). "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". J. Clin. Oncol. 23 (36): 9198-207. doi:10.1200/JCO.2005.02.907. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 16172458.  
  13. ^ Upfal A (2005). "Jane Austen’s lifelong health problems and final illness: New evidence points to a fatal Hodgkin’s disease and excludes the widely accepted Addison's". J Med Ethics Med Humanities 31: 3-11.  


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Hodgkin's lymphoma

-noun

  1. (pathology, oncology) A malignant lymphoid neoplasm characterized by the formation of large tumour cells (Reed-Sternberg cells) in the lymph nodes.
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