Hodgkin's lymphoma
From Wikipedia, the free encyclopedia
Jump to: navigation, search
Hodgkin's lymphoma
Classification and external resources
Micrograph showing Hodgkin's lymphoma (Field stain).
ICD-10 C81.
ICD-9 201
ICD-O: 9650/3-9667/3
DiseasesDB 5973
MedlinePlus 000580
eMedicine med/1022
MeSH D006689
Hodgkin's lymphoma, previously known as Hodgkin's disease, is a type of lymphoma, which is a cancer originating from white blood cells called lymphocytes. It was named after Thomas Hodgkin, who first described abnormalities in the lymph system in 1832.[1][2] Hodgkin's lymphoma is characterized by the orderly spread of disease from one lymph node group to another and by the development of systemic symptoms with advanced disease. When Hodgkins cells are examined microscopically, multinucleated Reed-Sternberg cells (RS cells) are the characteristic histopathologic finding. Hodgkin's lymphoma may be treated with radiation therapy, chemotherapy or hematopoietic stem cell transplantation, the choice of treatment depending on the age and sex of the patient and the stage, bulk and histological subtype of the disease.
The disease occurrence shows two peaks: the first in young adulthood (age 15–35) and the second in those over 55 years old.[3]
The 10-year overall survival rate is more than 90% for any stages, early stage may help more (stage I or II) Hodgkin's lymphoma. Since many patients are young, they often live 40 years or more after treatment. However, few studies follow patients as long as 25 years, and those studies are of older treatments with more life-threatening adverse effects, so it is impossible to predict long-term outcomes of newer, less harmful treatments. Radiation treatments, and some chemotherapy drugs, pose a risk of causing potentially fatal secondary cancers, heart disease, and lung disease 40 or more years later. Modern treatments greatly minimize the chances of these late effects.[4]
Patients with a history of infectious mononucleosis due to Epstein-Barr virus may have an increased risk of HL.[citation needed]
Contents
[hide]
* 1 Classification
o 1.1 Types
o 1.2 Staging
* 2 Signs and symptoms
* 3 Cause
* 4 Pathogenesis
* 5 Diagnosis
o 5.1 Pathology
* 6 Management
* 7 Prognosis
* 8 Epidemiology
* 9 History
* 10 Society and culture
o 10.1 Notable cases
* 11 See also
* 12 Further reading
* 13 References
* 14 External links
[edit] Classification
[edit] Types
Classical Hodgkin's lymphoma (excluding nodular lymphocyte predominant Hodgkin's lymphoma) 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 (the cell composition around the Reed-Sternberg cell(s)).
Name Description ICD-10 ICD-O
Nodular sclerosing CHL Is the most common subtype and is composed of large tumor nodules showing scattered lacunar classical RS cells set in a background of reactive lymphocytes, eosinophils and plasma cells with varying degrees of collagen fibrosis/sclerosis. C81.1 M9663/3
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. without sclerosis. This type is most often associated with EBV infection and may be confused with the early, so-called 'cellular' phase of nodular sclerosing CHL. C81.2 M9652/3.
Lymphocyte-rich or Lymphocytic predominance Is a rare subtype, show many features which may cause diagnostic confusion with nodular lymphocyte predominant B-cell Non-Hodgkin's Lymphoma (B-NHL). This form also has the most favorable prognosis. C81.0 M9651/3
Lymphocyte depleted Is a rare subtype, composed of large numbers of often pleomorphic RS cells with only few reactive lymphocytes which may easily be confused with diffuse large cell lymphoma. Many cases previously classified within this category would now be reclassified under anaplastic large cell lymphoma.[5] C81.3 M9653/3
Unspecified C81.9 M9650/3
Lymph node biopsy showing Hodgkin's lymphoma, mixed-cellularity type.
Nodular lymphocyte predominant Hodgkin's lymphoma expresses CD20, and is not currently considered a form of classical Hodgkin's.
For the other forms, although the traditional B cell markers (such as CD20) are not expressed on all cells,[5] Reed-Sternberg cells are usually of B cell origin.[6][7] Although Hodgkin's is now frequently grouped with other B cell malignancies, some T cell markers (such as CD2 and CD4) are occasionally expressed.[8] However, this may be an artifact of the ambiguity inherent in the diagnosis.
Hodgkin's cells produce Interleukin-21 (IL-21), which was once thought to be exclusive to T cells. This feature may explain the behavior of classical Hodgkin's lymphoma, including clusters of other immune cells gathered around HL cells (infiltrate) in cultures.[9]
[edit] Staging
CT image of a 46 year old patient with Hodgkin's lymphoma, image at neck height. On the left side of the patient's neck enlarged lymph nodes are visible (marked in red).
The staging is the same for both Hodgkin's as well as non-Hodgkin's lymphomas.
After Hodgkin's lymphoma is diagnosed, a patient will be staged: that is, they will undergo a series of tests and procedures that 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.
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):
* Stage I is involvement of a single lymph node region (I) (mostly the cervical region) or single extralymphatic site (Ie);
* Stage II is involvement of two or more lymph node regions on the same side of the diaphragm (II) or of one lymph node region and a contiguous extralymphatic site (IIe);
* 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);
* Stage IV is disseminated involvement of one or more extralymphatic organs.
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. For localized extranodal extension from mass of nodes that does not advance the stage, subscript 'E' is added.
[edit] Signs and symptoms
Patients with Hodgkin's lymphoma may present with the following symptoms:
* Night Sweats
* Unexplained weight loss
* Lymph nodes: the most common symptom of Hodgkin's is the painless enlargement of one or more lymph nodes. The nodes may also feel rubbery and swollen when examined. The nodes of the neck and shoulders (cervical and supraclavicular) are most frequently involved (80–90% of the time, on average). The lymph nodes of the chest are often affected, and these may be noticed on a chest radiograph.
* Splenomegaly: enlargement of the spleen occurs in about 30% of people with Hodgkin's lymphoma. The enlargement, however, is seldom massive and the size of the spleen may fluctuate during the course of treatment.
* Hepatomegaly: enlargement of the liver, due to liver involvement, is present in about 5% of cases.
* Hepatosplenomegaly: the enlargement of both the liver and spleen caused by the same disease.
* Pain following alcohol consumption: classically, involved nodes are painful after alcohol consumption, though this phenomenon is very uncommon.[10]
* Back pain: nonspecific back pain (pain that cannot be localized or its cause determined by examination or scanning techniques) has been reported in some cases of Hodgkin's lymphoma. The lower back is most often affected.[citation needed]
* Red-coloured patches on the skin, easy bleeding and petechiae due to low platelet count (as a result of bone marrow infiltration, increased trapping in the spleen etc. – i.e. decreased production, increased removal)
* Systemic symptoms: about one-third of patients with Hodgkin's disease may also present with systemic symptoms, including low-grade fever; night sweats; unexplained weight loss of at least 10% of the patient's total body mass in six months or less, itchy skin (pruritus) due to increased levels of eosinophils in the bloodstream; or fatigue (lassitude). Systemic symptoms such as fever, night sweats, and weight loss are known as B symptoms; thus, presence of fever, weight loss, and night sweats indicate that the patient's stage is, for example, 2B instead of 2A.[11]
* Cyclical fever: patients may also present with a cyclical high-grade fever known as the Pel-Ebstein fever,[12] or more simply "P-E fever". However, there is debate as to whether or not the P-E fever truly exists.[13]
[edit] Cause
There are no guidelines for preventing Hodgkin's lymphoma because the cause is unknown or multifactorial. A risk factor is something that statistically increases your chance of getting a disease or condition. Risk factors include:
* Sex: male[14]
* Ages: 15–40 and over 55[14]
* Family history[14]
* History of infectious mononucleosis or infection with Epstein-Barr virus, a causative agent of mononucleosis[14]
* Weakened immune system, including infection with HIV or the presence of AIDS[14]
* Prolonged use of human growth hormone[14]
* Exotoxins, such as Agent Orange
[edit] Pathogenesis
Mind map 3.jpg
[edit] Diagnosis
This section does not cite any references or sources.
Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed. (January 2009)
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). Blood tests 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. PET scans are also useful in functional imaging (by using a radiolabeled glucose to image tissues of high metabolism). In some cases a Gallium Scan may be used instead of a PET scan.
[edit] 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.
A fibrin ring granuloma may be seen.
Microscopy
Micrograph of a classic Reed-Sternberg cell.
Micrograph showing a "popcorn cell", the Reed-Sternberg cell variant seen in nodular lymphocyte predominant Hodgkin lymphoma.H&E stain.
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 (RSC) (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.
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).
Variants:
* Hodgkin cell (atypical mononuclear RSC) is a variant of RS cell, which has the same characteristics, but is mononucleated.
* 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").
* Pleomorphic RSC has multiple irregular nuclei.
* "Popcorn" RSC (lympho-histiocytic variant) is a small cell, with a very lobulated nucleus, small nucleoli.
* "Mummy" RSC has a compact nucleus, no nucleolus and basophilic cytoplasm.
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 classic Hodgkin's lymphoma usually depends on the stage of disease rather than the histotype.
[edit] Management
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.
From Wikipedia, the free encyclopedia
Jump to: navigation, search
Hodgkin's lymphoma
Classification and external resources
Micrograph showing Hodgkin's lymphoma (Field stain).
ICD-10 C81.
ICD-9 201
ICD-O: 9650/3-9667/3
DiseasesDB 5973
MedlinePlus 000580
eMedicine med/1022
MeSH D006689
Hodgkin's lymphoma, previously known as Hodgkin's disease, is a type of lymphoma, which is a cancer originating from white blood cells called lymphocytes. It was named after Thomas Hodgkin, who first described abnormalities in the lymph system in 1832.[1][2] Hodgkin's lymphoma is characterized by the orderly spread of disease from one lymph node group to another and by the development of systemic symptoms with advanced disease. When Hodgkins cells are examined microscopically, multinucleated Reed-Sternberg cells (RS cells) are the characteristic histopathologic finding. Hodgkin's lymphoma may be treated with radiation therapy, chemotherapy or hematopoietic stem cell transplantation, the choice of treatment depending on the age and sex of the patient and the stage, bulk and histological subtype of the disease.
The disease occurrence shows two peaks: the first in young adulthood (age 15–35) and the second in those over 55 years old.[3]
The 10-year overall survival rate is more than 90% for any stages, early stage may help more (stage I or II) Hodgkin's lymphoma. Since many patients are young, they often live 40 years or more after treatment. However, few studies follow patients as long as 25 years, and those studies are of older treatments with more life-threatening adverse effects, so it is impossible to predict long-term outcomes of newer, less harmful treatments. Radiation treatments, and some chemotherapy drugs, pose a risk of causing potentially fatal secondary cancers, heart disease, and lung disease 40 or more years later. Modern treatments greatly minimize the chances of these late effects.[4]
Patients with a history of infectious mononucleosis due to Epstein-Barr virus may have an increased risk of HL.[citation needed]
Contents
[hide]
* 1 Classification
o 1.1 Types
o 1.2 Staging
* 2 Signs and symptoms
* 3 Cause
* 4 Pathogenesis
* 5 Diagnosis
o 5.1 Pathology
* 6 Management
* 7 Prognosis
* 8 Epidemiology
* 9 History
* 10 Society and culture
o 10.1 Notable cases
* 11 See also
* 12 Further reading
* 13 References
* 14 External links
[edit] Classification
[edit] Types
Classical Hodgkin's lymphoma (excluding nodular lymphocyte predominant Hodgkin's lymphoma) 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 (the cell composition around the Reed-Sternberg cell(s)).
Name Description ICD-10 ICD-O
Nodular sclerosing CHL Is the most common subtype and is composed of large tumor nodules showing scattered lacunar classical RS cells set in a background of reactive lymphocytes, eosinophils and plasma cells with varying degrees of collagen fibrosis/sclerosis. C81.1 M9663/3
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. without sclerosis. This type is most often associated with EBV infection and may be confused with the early, so-called 'cellular' phase of nodular sclerosing CHL. C81.2 M9652/3.
Lymphocyte-rich or Lymphocytic predominance Is a rare subtype, show many features which may cause diagnostic confusion with nodular lymphocyte predominant B-cell Non-Hodgkin's Lymphoma (B-NHL). This form also has the most favorable prognosis. C81.0 M9651/3
Lymphocyte depleted Is a rare subtype, composed of large numbers of often pleomorphic RS cells with only few reactive lymphocytes which may easily be confused with diffuse large cell lymphoma. Many cases previously classified within this category would now be reclassified under anaplastic large cell lymphoma.[5] C81.3 M9653/3
Unspecified C81.9 M9650/3
Lymph node biopsy showing Hodgkin's lymphoma, mixed-cellularity type.
Nodular lymphocyte predominant Hodgkin's lymphoma expresses CD20, and is not currently considered a form of classical Hodgkin's.
For the other forms, although the traditional B cell markers (such as CD20) are not expressed on all cells,[5] Reed-Sternberg cells are usually of B cell origin.[6][7] Although Hodgkin's is now frequently grouped with other B cell malignancies, some T cell markers (such as CD2 and CD4) are occasionally expressed.[8] However, this may be an artifact of the ambiguity inherent in the diagnosis.
Hodgkin's cells produce Interleukin-21 (IL-21), which was once thought to be exclusive to T cells. This feature may explain the behavior of classical Hodgkin's lymphoma, including clusters of other immune cells gathered around HL cells (infiltrate) in cultures.[9]
[edit] Staging
CT image of a 46 year old patient with Hodgkin's lymphoma, image at neck height. On the left side of the patient's neck enlarged lymph nodes are visible (marked in red).
The staging is the same for both Hodgkin's as well as non-Hodgkin's lymphomas.
After Hodgkin's lymphoma is diagnosed, a patient will be staged: that is, they will undergo a series of tests and procedures that 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.
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):
* Stage I is involvement of a single lymph node region (I) (mostly the cervical region) or single extralymphatic site (Ie);
* Stage II is involvement of two or more lymph node regions on the same side of the diaphragm (II) or of one lymph node region and a contiguous extralymphatic site (IIe);
* 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);
* Stage IV is disseminated involvement of one or more extralymphatic organs.
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. For localized extranodal extension from mass of nodes that does not advance the stage, subscript 'E' is added.
[edit] Signs and symptoms
Patients with Hodgkin's lymphoma may present with the following symptoms:
* Night Sweats
* Unexplained weight loss
* Lymph nodes: the most common symptom of Hodgkin's is the painless enlargement of one or more lymph nodes. The nodes may also feel rubbery and swollen when examined. The nodes of the neck and shoulders (cervical and supraclavicular) are most frequently involved (80–90% of the time, on average). The lymph nodes of the chest are often affected, and these may be noticed on a chest radiograph.
* Splenomegaly: enlargement of the spleen occurs in about 30% of people with Hodgkin's lymphoma. The enlargement, however, is seldom massive and the size of the spleen may fluctuate during the course of treatment.
* Hepatomegaly: enlargement of the liver, due to liver involvement, is present in about 5% of cases.
* Hepatosplenomegaly: the enlargement of both the liver and spleen caused by the same disease.
* Pain following alcohol consumption: classically, involved nodes are painful after alcohol consumption, though this phenomenon is very uncommon.[10]
* Back pain: nonspecific back pain (pain that cannot be localized or its cause determined by examination or scanning techniques) has been reported in some cases of Hodgkin's lymphoma. The lower back is most often affected.[citation needed]
* Red-coloured patches on the skin, easy bleeding and petechiae due to low platelet count (as a result of bone marrow infiltration, increased trapping in the spleen etc. – i.e. decreased production, increased removal)
* Systemic symptoms: about one-third of patients with Hodgkin's disease may also present with systemic symptoms, including low-grade fever; night sweats; unexplained weight loss of at least 10% of the patient's total body mass in six months or less, itchy skin (pruritus) due to increased levels of eosinophils in the bloodstream; or fatigue (lassitude). Systemic symptoms such as fever, night sweats, and weight loss are known as B symptoms; thus, presence of fever, weight loss, and night sweats indicate that the patient's stage is, for example, 2B instead of 2A.[11]
* Cyclical fever: patients may also present with a cyclical high-grade fever known as the Pel-Ebstein fever,[12] or more simply "P-E fever". However, there is debate as to whether or not the P-E fever truly exists.[13]
[edit] Cause
There are no guidelines for preventing Hodgkin's lymphoma because the cause is unknown or multifactorial. A risk factor is something that statistically increases your chance of getting a disease or condition. Risk factors include:
* Sex: male[14]
* Ages: 15–40 and over 55[14]
* Family history[14]
* History of infectious mononucleosis or infection with Epstein-Barr virus, a causative agent of mononucleosis[14]
* Weakened immune system, including infection with HIV or the presence of AIDS[14]
* Prolonged use of human growth hormone[14]
* Exotoxins, such as Agent Orange
[edit] Pathogenesis
Mind map 3.jpg
[edit] Diagnosis
This section does not cite any references or sources.
Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed. (January 2009)
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). Blood tests 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. PET scans are also useful in functional imaging (by using a radiolabeled glucose to image tissues of high metabolism). In some cases a Gallium Scan may be used instead of a PET scan.
[edit] 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.
A fibrin ring granuloma may be seen.
Microscopy
Micrograph of a classic Reed-Sternberg cell.
Micrograph showing a "popcorn cell", the Reed-Sternberg cell variant seen in nodular lymphocyte predominant Hodgkin lymphoma.H&E stain.
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 (RSC) (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.
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).
Variants:
* Hodgkin cell (atypical mononuclear RSC) is a variant of RS cell, which has the same characteristics, but is mononucleated.
* 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").
* Pleomorphic RSC has multiple irregular nuclei.
* "Popcorn" RSC (lympho-histiocytic variant) is a small cell, with a very lobulated nucleus, small nucleoli.
* "Mummy" RSC has a compact nucleus, no nucleolus and basophilic cytoplasm.
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 classic Hodgkin's lymphoma usually depends on the stage of disease rather than the histotype.
[edit] Management
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.
