Hodgkin's lymphoma, Hodgkin's disease also known as a type of lymphoma first described by Thomas Hodgkin in 1832. Hodgkin's lymphoma is characterized clinically by the orderly spread of disease to a group of lymph nodes to the other, marked the development of systemic symptoms with advanced disease. Histologically the disease is characterized by the presence of Reed-Sternberg cells (RS cells). Hodgkin's lymphoma is a cancer of the first to be cured by radiation. Later he became one of the first to be cured by chemotherapy. The survival rate is usually around 90% if the disease is detected early, making it one of the most curable forms of cancer. Recent studies show survival rates five years that have seen up to 98% of patients in early stages. Later-stage cancer, but still significantly worse prognosis. Complete remission (cure) of stages I and II is about 85 to 90%, whereas in stages III and IV, the cure rate is 80%.
Epidemiology
Unlike some other lymphomas, whose incidence increases with age, Hodgkin's lymphoma has a bimodal incidence curve separately, so it is most common in two age groups, the first adult to young (15-35 years) and the second at the age of 55 years, although these peaks may vary slightly depending on nationality. In general, it is more common in men, with the exception of the nodular sclerosis variant, which is more common in women.
The annual incidence of Hodgkin's lymphoma is one of 25,000 people, and the disease less than 1% of all cancers worldwide accounts.
The incidence of Hodgkin's lymphoma in patients infected with HIV increased. Unlike many other associated lymphomas in HIV infection is more common in patients with a higher number of CD4 + T cells.
Symptoms
Patients with Hodgkin's lymphoma can cause the following symptoms:
Night sweats Lymph nodes: the most common symptom of lymphoma is painless enlargement of one or more lymph nodes. The nodes may also feel swollen gum and when examined by a doctor. Glands in the neck and shoulders (cervical and supra) are frequently affected (80-90% of the time, on average). The lymph nodes in the chest are often affected and can be found on a radiograph. Splenomegaly occurs enlarged spleen in about 30% of people with Hodgkin's disease. The extension is rare and massive spleen may fluctuate during the treatment. Hepatomegaly: enlargement of the liver due to liver disease and is about five percent of cases. Hepatosplenomegaly: Enlargement of the liver and spleen caused by the disease. Pain: Pain after consuming alcohol: Classical, involved nodes are painful after alcohol consumption, although this is rare. Back pain: nonspecific back pain (pain that can not be removed or if a test determines her or scanning techniques) have reported some cases of Hodgkin's lymphoma. The lower back is most often affected. red spots on the skin, easy bruising and petechiae due to low platelet count Systemic symptoms: about one-third (⅓) of patients with Hodgkin's disease can also cause systemic symptoms such as mild fever, night sweats, unexplained weight loss of at least 10% of the total mass of the body of the patient in six months or less, itching (pruritus) due to the higher levels of eosinophils in the blood, or fatigue (tiredness). Systemic symptoms such as fever, night sweats and weight loss are known as B symptoms, so that the presence of fever, sweats, weight loss and night at this stage of the patient, for example, instead of 2A 2B. cyclic fever may, patients also have a high fever known as cyclic Pel-Ebstein fever, or just "fever EP." However, there is debate about whether a fever is PE. Risk Factors
There are no guidelines for the prevention of Hodgkin's disease, because the cause is unknown. A risk factor is something that your chances of getting a disease or increases.
Among the risk factors:
Gender: Male Ages: 15-40 and over 55 years Family history History of infectious mononucleosis or infection with the Epstein-Barr virus, the cause of mononucleosis An immune deficiency, including infection with HIV or AIDS presence Prolonged use of human growth hormone
Diagnosis
Hodgkin's lymphoma must be distinguished from non-cancer causes swollen lymph nodes (for example, various infections) and other cancers. The definitive diagnosis is by biopsy of lymph nodes (usually a surgical biopsy with microscopic examination). Blood tests are also performed to assess organ function and to evaluate the safety of chemotherapy. Positron emission tomography (PET) is used to small deposits, which record not on the scanner. In some cases, gallium scan can be used instead of a PET scan.
Pathology
Macroscopic
Lymph nodes (most often the lateral neck lymph nodes) are enlarged, but the shape is preserved because the capsule is not penetrated. Normally, the cut surface gray-white uniform, in some histological subtypes (eg nodular sclerosis) may show a nodular appearance.
Microscopy
Microscopic examination of the node biopsy shows a full or partial cancellation of the lymph node architecture by scattered large malignant cells called Reed-Sternberg cells (typical and variants) within a cell reactive added to the variable portions are infiltrating lymphocytes, histiocytes, eosinophils and plasma cells . The Reed-Sternberg cells are large cells than bi-nucleated often with prominent nucleoli and some CD45, CD30 +, CD15 + / identified - immunophenotype. In about 50% of cases, the Reed-Sternberg cells with the Epstein-Barr virus-infected.
Properties of the Reed-Sternberg cells of classic include large (20-50 microns), abundant amphiphilic finely granular / homogeneous cytoplasm, two mirror-image nuclei (owl eyes) each with an eosinophilic nucleolus and a thick nuclear membrane (chromatin is in the periphery of the cell) distributed.
Variations:
Lymphoma (atypical mononuclear RSC) is a variant of RS cell, which has the same features, but it is mononuclear. Lacunar RSC is large with a single core hyperlobated, multiple nucleoli, eosinophilic cytoplasm and small leaves at the core, creating a space ("gap"). Pleomorphic RSC has several irregular nuclei. "Popcorn" RSC (lympho histiocytic variant) is a small cell with a nucleus of very bumpy, small nucleoli. "Mama," RSC has a compact nucleus, no nucleolus and basophilic cytoplasm. Hodgkin's lymphoma can be sub-classified according to histological type. The histology of Hodgkin's lymphoma is not as important as in non-Hodgkin's lymphoma: treatment and prognosis of Hodgkin's disease depend on the stage of the disease rather than the histotype.
Types
classical Hodgkin's lymphoma (excluding nodular lymphocyte predominant Hodgkin's) can occur in 4 subtypes on the pathological morphology of the Reed-Sternberg cells and the composition of the reactive cells are classified in the biopsy of the lymph node infiltrate seen.
Description Name
Nodular sclerosing classic LCH is the most common form and is composed of large tumor nodules with lacunar RS cells subtype of numerous classic often pleomorphic RS cells with very few reactive lymphocytes which may easily be confused with lymphoma, there is diffuse large cell cells.
Classic subtype of mixed cellularity subtype is a common and consists of numerous classic RS cells with many inflammatory cells, including lymphocytes, histiocytes, eosinophils, plasma cells, y. added
Lymphocyte-rich classic is a rare subspecies.
Classic depleted cells is a rare subspecies.
The cell non-classical nodular lymphocyte predominant Hodgkin (NLPHL) is no more than a form of classical Hodgkin's lymphoma. For the RSC variants (popcorn cells) that characterize this form of the disease always express B lymphocyte markers such as CD20 (ie NLPHL an unusual form of B-cell lymphoma), and (in contrast to classic HL) Progress NLPHL todiffuse large B-cell lymphoma There are small differences, but also in the prognosis of different forms. Lymphocyte predominant HL is a rare subspecies of vague nodules of numerous reactive lymphocytes with large popcorn-shaped RS together added. Unlike classic RSC, the non-classic popcorn-RS cells as CD15 and CD30 NLPHL negative, while positive for the marker CD20 on B cells
Despite not express the traditional markers of B-cells, the Reed-Sternberg cells usually B-cell origin
Staging
After Hodgkin's disease is diagnosed, the patient will take place: it is, with a series of tests and procedures carried out to determine areas of the body are affected. These procedures include documentation of their histology, a physical examination, blood tests, X-rays, X-ray computed tomography (CT) or magnetic resonance imaging (MRI) of the chest, abdomen and pelvis and a bone marrow biopsy. Positron emission tomography (PET) is now used instead of gallium scintigraphy in the assessment. In the past, lymphography and surgical laparotomy (which involves opening the abdomen, and visual inspection of the tumors) were performed. Lymphangiography and laparotomy are very rarely played, it was supplanted by the image enhancement on CT and PET.
On the basis of this staging, the patient should be classified according to staging (Ann Arbor staging a common classification system):
Level I involvement of regional lymph nodes (I) or a single site is extralymphatic (IE) The second step is the participation of two or more lymph node regions on the same side of the diaphragm (II) or regional lymph nodes and a site adjacent extralymphatic (IIE) Level III is the involvement of lymph node regions on both sides of the membrane, which may involve the spleen (IIIS) and / or limited contiguous organ or extralymphatic site (IIIe, IIIes) Stage IV is the involvement of one or more organs extralymphatic common. The absence of systemic symptoms by the addition of "A" is referred to in the scene is the presence of systemic symptoms by the addition of "B" meant for the stage.
For localized extranodal extension of the mass of the nodes that do not advance not the stage, the index "E" is added.
WEATHER
The treatment of Hodgkin's disease in recent decades improved. Recent studies have created new types of chemotherapy showed higher survival rates, which were shown before. In a recent European study, the survival rate after 5 years in patients with a favorable prognosis 98%, while patients with less chance of at least 85%.
In 1998, an international effort identified seven prognostic factors that accurately predict the success rate of conventional treatment in patients with advanced stage Hodgkin's lymphoma, local or wide. The freedom of progression (FFP) at 5 years was directly on the number of factors used in one patient. The 5-year FFP for patients with zero factors is 84%. Any other factor that reduces the deposition rate for 5 years at 7%, while 5-year FFP for patients with factors of 5 or more is 42%.
Adverse prognostic factors in the international study are identified:
Age> = 45 years Stage IV Hemoglobin <> Lymphocyte count <> Male Albumin <> WBC count> = 15,000 / microliter Other studies have reported that the following are the main negative prognostic factors: mixed cellularity or lymphocyte depleted histologies, male sex, number of nodes involved sites, advanced stage, age 40 years or more, the presence of B symptoms, high sedimentation and tumor burden (widening of the mediastinum by more than a third, or the presence of a larger node degree than 10 cm in any dimension.)
Treatment
Patients with early stage disease (IA or IIA) are effectively treated by radiotherapy or chemotherapy. The choice of treatment depends on age, sex, bulk and histological subtypes of the disease. Patients with later disease (III, IVA or IVB) are alone with a combination chemotherapy. Patients in each stage with a large mass in the chest are usually treated with a combination of chemotherapy and radiotherapy.
Currently, the ABVD chemotherapy, the gold standard for the treatment of Hodgkin's disease. The initials of the four drugs Adriamycin, bleomycin, vinblastine and dacarbazine match. Developed in Italy in the 1970s, the ABVD treatment typically takes six to eight months, though more treatments are required. Another form of treatment is the latest Stanford V regime, which usually contains only half as long as the ABVD but intensive chemotherapy schedule and includes irradiation. But in a randomized controlled trial was inferior to Stanford V.
Another form of treatment, particularly in Europe for stages> II is BEACOPP. The cure rate with the BEACOPP ESC. Scheme is about 10-15% higher than standard ABVD in advanced stages. Although it is shown in a reference document in the New England Journal of Medicine was (Diehl et al.) U.S. physicians still favor ABVD. Probably because some doctors think that BEACOPP triggers several secondary leukemia. However, this seems insignificant compared to the higher cure rates. Also, BEACOPP is more expensive because of the need for accompanying G-CSF to increase white blood cells. To test the German Hodgkin Study Group 8 cycles (8x) BEACOPP esc vs. 6x BEACOPP esc vs. 8x BEACOPP-14 baseline (HD15-test).
The high cure rate and long survival of many patients with Hodgkin's lymphoma is a major concern in the late treatment effects, including cardiovascular disease and secondary malignancies such as acute leukemia, lymphomas and solid tumors, radiation therapy. Most patients in the early stages of the disease with chemotherapy and radiation, the shorter it is treated in the field, rather than radiation therapy alone. Clinical research strategies are being investigated to reduce the duration of chemotherapy and dose and volume of radiation therapy to reduce morbidity and mortality by the end of treatment and at the same high cure rates. Hospitals are also those who respond quickly to treatment with chemotherapy without radiotherapy.
Epidemiology
Unlike some other lymphomas, whose incidence increases with age, Hodgkin's lymphoma has a bimodal incidence curve separately, so it is most common in two age groups, the first adult to young (15-35 years) and the second at the age of 55 years, although these peaks may vary slightly depending on nationality. In general, it is more common in men, with the exception of the nodular sclerosis variant, which is more common in women.
The annual incidence of Hodgkin's lymphoma is one of 25,000 people, and the disease less than 1% of all cancers worldwide accounts.
The incidence of Hodgkin's lymphoma in patients infected with HIV increased. Unlike many other associated lymphomas in HIV infection is more common in patients with a higher number of CD4 + T cells.
Symptoms
Patients with Hodgkin's lymphoma can cause the following symptoms:
Night sweats Lymph nodes: the most common symptom of lymphoma is painless enlargement of one or more lymph nodes. The nodes may also feel swollen gum and when examined by a doctor. Glands in the neck and shoulders (cervical and supra) are frequently affected (80-90% of the time, on average). The lymph nodes in the chest are often affected and can be found on a radiograph. Splenomegaly occurs enlarged spleen in about 30% of people with Hodgkin's disease. The extension is rare and massive spleen may fluctuate during the treatment. Hepatomegaly: enlargement of the liver due to liver disease and is about five percent of cases. Hepatosplenomegaly: Enlargement of the liver and spleen caused by the disease. Pain: Pain after consuming alcohol: Classical, involved nodes are painful after alcohol consumption, although this is rare. Back pain: nonspecific back pain (pain that can not be removed or if a test determines her or scanning techniques) have reported some cases of Hodgkin's lymphoma. The lower back is most often affected. red spots on the skin, easy bruising and petechiae due to low platelet count Systemic symptoms: about one-third (⅓) of patients with Hodgkin's disease can also cause systemic symptoms such as mild fever, night sweats, unexplained weight loss of at least 10% of the total mass of the body of the patient in six months or less, itching (pruritus) due to the higher levels of eosinophils in the blood, or fatigue (tiredness). Systemic symptoms such as fever, night sweats and weight loss are known as B symptoms, so that the presence of fever, sweats, weight loss and night at this stage of the patient, for example, instead of 2A 2B. cyclic fever may, patients also have a high fever known as cyclic Pel-Ebstein fever, or just "fever EP." However, there is debate about whether a fever is PE. Risk Factors
There are no guidelines for the prevention of Hodgkin's disease, because the cause is unknown. A risk factor is something that your chances of getting a disease or increases.
Among the risk factors:
Gender: Male Ages: 15-40 and over 55 years Family history History of infectious mononucleosis or infection with the Epstein-Barr virus, the cause of mononucleosis An immune deficiency, including infection with HIV or AIDS presence Prolonged use of human growth hormone
Diagnosis
Hodgkin's lymphoma must be distinguished from non-cancer causes swollen lymph nodes (for example, various infections) and other cancers. The definitive diagnosis is by biopsy of lymph nodes (usually a surgical biopsy with microscopic examination). Blood tests are also performed to assess organ function and to evaluate the safety of chemotherapy. Positron emission tomography (PET) is used to small deposits, which record not on the scanner. In some cases, gallium scan can be used instead of a PET scan.
Pathology
Macroscopic
Lymph nodes (most often the lateral neck lymph nodes) are enlarged, but the shape is preserved because the capsule is not penetrated. Normally, the cut surface gray-white uniform, in some histological subtypes (eg nodular sclerosis) may show a nodular appearance.
Microscopy
Microscopic examination of the node biopsy shows a full or partial cancellation of the lymph node architecture by scattered large malignant cells called Reed-Sternberg cells (typical and variants) within a cell reactive added to the variable portions are infiltrating lymphocytes, histiocytes, eosinophils and plasma cells . The Reed-Sternberg cells are large cells than bi-nucleated often with prominent nucleoli and some CD45, CD30 +, CD15 + / identified - immunophenotype. In about 50% of cases, the Reed-Sternberg cells with the Epstein-Barr virus-infected.
Properties of the Reed-Sternberg cells of classic include large (20-50 microns), abundant amphiphilic finely granular / homogeneous cytoplasm, two mirror-image nuclei (owl eyes) each with an eosinophilic nucleolus and a thick nuclear membrane (chromatin is in the periphery of the cell) distributed.
Variations:
Lymphoma (atypical mononuclear RSC) is a variant of RS cell, which has the same features, but it is mononuclear. Lacunar RSC is large with a single core hyperlobated, multiple nucleoli, eosinophilic cytoplasm and small leaves at the core, creating a space ("gap"). Pleomorphic RSC has several irregular nuclei. "Popcorn" RSC (lympho histiocytic variant) is a small cell with a nucleus of very bumpy, small nucleoli. "Mama," RSC has a compact nucleus, no nucleolus and basophilic cytoplasm. Hodgkin's lymphoma can be sub-classified according to histological type. The histology of Hodgkin's lymphoma is not as important as in non-Hodgkin's lymphoma: treatment and prognosis of Hodgkin's disease depend on the stage of the disease rather than the histotype.
Types
classical Hodgkin's lymphoma (excluding nodular lymphocyte predominant Hodgkin's) can occur in 4 subtypes on the pathological morphology of the Reed-Sternberg cells and the composition of the reactive cells are classified in the biopsy of the lymph node infiltrate seen.
Description Name
Nodular sclerosing classic LCH is the most common form and is composed of large tumor nodules with lacunar RS cells subtype of numerous classic often pleomorphic RS cells with very few reactive lymphocytes which may easily be confused with lymphoma, there is diffuse large cell cells.
Classic subtype of mixed cellularity subtype is a common and consists of numerous classic RS cells with many inflammatory cells, including lymphocytes, histiocytes, eosinophils, plasma cells, y. added
Lymphocyte-rich classic is a rare subspecies.
Classic depleted cells is a rare subspecies.
The cell non-classical nodular lymphocyte predominant Hodgkin (NLPHL) is no more than a form of classical Hodgkin's lymphoma. For the RSC variants (popcorn cells) that characterize this form of the disease always express B lymphocyte markers such as CD20 (ie NLPHL an unusual form of B-cell lymphoma), and (in contrast to classic HL) Progress NLPHL todiffuse large B-cell lymphoma There are small differences, but also in the prognosis of different forms. Lymphocyte predominant HL is a rare subspecies of vague nodules of numerous reactive lymphocytes with large popcorn-shaped RS together added. Unlike classic RSC, the non-classic popcorn-RS cells as CD15 and CD30 NLPHL negative, while positive for the marker CD20 on B cells
Despite not express the traditional markers of B-cells, the Reed-Sternberg cells usually B-cell origin
Staging
After Hodgkin's disease is diagnosed, the patient will take place: it is, with a series of tests and procedures carried out to determine areas of the body are affected. These procedures include documentation of their histology, a physical examination, blood tests, X-rays, X-ray computed tomography (CT) or magnetic resonance imaging (MRI) of the chest, abdomen and pelvis and a bone marrow biopsy. Positron emission tomography (PET) is now used instead of gallium scintigraphy in the assessment. In the past, lymphography and surgical laparotomy (which involves opening the abdomen, and visual inspection of the tumors) were performed. Lymphangiography and laparotomy are very rarely played, it was supplanted by the image enhancement on CT and PET.
On the basis of this staging, the patient should be classified according to staging (Ann Arbor staging a common classification system):
Level I involvement of regional lymph nodes (I) or a single site is extralymphatic (IE) The second step is the participation of two or more lymph node regions on the same side of the diaphragm (II) or regional lymph nodes and a site adjacent extralymphatic (IIE) Level III is the involvement of lymph node regions on both sides of the membrane, which may involve the spleen (IIIS) and / or limited contiguous organ or extralymphatic site (IIIe, IIIes) Stage IV is the involvement of one or more organs extralymphatic common. The absence of systemic symptoms by the addition of "A" is referred to in the scene is the presence of systemic symptoms by the addition of "B" meant for the stage.
For localized extranodal extension of the mass of the nodes that do not advance not the stage, the index "E" is added.
WEATHER
The treatment of Hodgkin's disease in recent decades improved. Recent studies have created new types of chemotherapy showed higher survival rates, which were shown before. In a recent European study, the survival rate after 5 years in patients with a favorable prognosis 98%, while patients with less chance of at least 85%.
In 1998, an international effort identified seven prognostic factors that accurately predict the success rate of conventional treatment in patients with advanced stage Hodgkin's lymphoma, local or wide. The freedom of progression (FFP) at 5 years was directly on the number of factors used in one patient. The 5-year FFP for patients with zero factors is 84%. Any other factor that reduces the deposition rate for 5 years at 7%, while 5-year FFP for patients with factors of 5 or more is 42%.
Adverse prognostic factors in the international study are identified:
Age> = 45 years Stage IV Hemoglobin <> Lymphocyte count <> Male Albumin <> WBC count> = 15,000 / microliter Other studies have reported that the following are the main negative prognostic factors: mixed cellularity or lymphocyte depleted histologies, male sex, number of nodes involved sites, advanced stage, age 40 years or more, the presence of B symptoms, high sedimentation and tumor burden (widening of the mediastinum by more than a third, or the presence of a larger node degree than 10 cm in any dimension.)
Treatment
Patients with early stage disease (IA or IIA) are effectively treated by radiotherapy or chemotherapy. The choice of treatment depends on age, sex, bulk and histological subtypes of the disease. Patients with later disease (III, IVA or IVB) are alone with a combination chemotherapy. Patients in each stage with a large mass in the chest are usually treated with a combination of chemotherapy and radiotherapy.
Currently, the ABVD chemotherapy, the gold standard for the treatment of Hodgkin's disease. The initials of the four drugs Adriamycin, bleomycin, vinblastine and dacarbazine match. Developed in Italy in the 1970s, the ABVD treatment typically takes six to eight months, though more treatments are required. Another form of treatment is the latest Stanford V regime, which usually contains only half as long as the ABVD but intensive chemotherapy schedule and includes irradiation. But in a randomized controlled trial was inferior to Stanford V.
Another form of treatment, particularly in Europe for stages> II is BEACOPP. The cure rate with the BEACOPP ESC. Scheme is about 10-15% higher than standard ABVD in advanced stages. Although it is shown in a reference document in the New England Journal of Medicine was (Diehl et al.) U.S. physicians still favor ABVD. Probably because some doctors think that BEACOPP triggers several secondary leukemia. However, this seems insignificant compared to the higher cure rates. Also, BEACOPP is more expensive because of the need for accompanying G-CSF to increase white blood cells. To test the German Hodgkin Study Group 8 cycles (8x) BEACOPP esc vs. 6x BEACOPP esc vs. 8x BEACOPP-14 baseline (HD15-test).
The high cure rate and long survival of many patients with Hodgkin's lymphoma is a major concern in the late treatment effects, including cardiovascular disease and secondary malignancies such as acute leukemia, lymphomas and solid tumors, radiation therapy. Most patients in the early stages of the disease with chemotherapy and radiation, the shorter it is treated in the field, rather than radiation therapy alone. Clinical research strategies are being investigated to reduce the duration of chemotherapy and dose and volume of radiation therapy to reduce morbidity and mortality by the end of treatment and at the same high cure rates. Hospitals are also those who respond quickly to treatment with chemotherapy without radiotherapy.
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