Angioimmunoblastic T-cell lymphoma overview: Difference between revisions
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==Overview== | ==Overview== | ||
Angioimmunoblastic T-cell lymphoma (AILT) is a mature [[T-cell]] [[lymphoma]] characterized by a polymorphous [[lymph node]] infiltrate showing a marked increase in [[follicular dendritic cells]] (FDCs) and [[high endothelial venules]] (HEVs). It is also known as immunoblastic lymphadenopathy (Lukes-Collins Classification) and AILD-type (lymphogranulomatosis X) T-cell lymphoma (Kiel Classification). Clonal [[T-cell receptor]] and [[immunoglobulin]] [[gene]] rearrangements are involved in the [[pathogenesis]] of angioimmunoblastic T-cell lymphoma. On [[gross pathology]], aborization and proliferation of [[high endothelial venules]] are characteristic findings of angioimmunoblastic T-cell lymphoma. On [[microscopic]] [[Histopathology|histopathological]] [[analysis]], [[CD4]]+ [[T-cells]], [[CD8]]+ [[T-cells]], and polyclonal [[plasma cells]] are characteristic findings of angioimmunoblastic T-cell lymphoma. There are no established causes for angioimmunoblastic T-cell lymphoma. Angioimmunoblastic T-cell lymphoma must be differentiated from other diseases such as peripheral T-cell lymphoma-not otherwise specified, [[classical Hodgkin's lymphoma]], [[diffuse large B cell lymphoma]], and [[nodal marginal zone lymphoma]]. Angioimmunoblastic T-cell lymphoma commonly affects middle-aged or elderly individuals. Angioimmunoblastic T-cell lymphoma affects men and women equally. There are no established risk factors for angioimmunoblastic T-cell lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for angioimmunoblastic T-cell lymphoma. Common [[Complication (medicine)|complications]] of angioimmunoblastic T-cell lymphoma include [[rheumatoid arthritis]], [[autoimmune hemolytic anemia]], [[vasculitis]], and [[Autoimmunity|autoimmune]] [[thyroid]] disease. According to the Lugano classification, there are four stages of angioimmunoblastic T-cell lymphoma based on the number of [[Lymph node|nodes]] and [[Lymph node|extranodal]] involvement. The most common [[Symptom|symptoms]] of angioimmunoblastic T-cell lymphoma include [[fever]], [[weight loss]], [[Rash|skin rash]], [[night sweats]], [[edema]], [[chest pain]], [[abdominal pain]], [[bone pain]], [[fatigue]], [[dark urine]], [[shortness of breath]], [[chronic pain]], [[Edema|swelling]] of [[Joint|joints]], and painless swelling of the [[neck]], [[axilla]], [[groin]], [[Chest|thorax]], and [[abdomen]]. Common [[physical examination]] findings of angioimmunoblastic T-cell lymphoma include [[fever]], [[rash]], [[ulcer]], [[splenomegaly]], [[hepatomegaly]], [[pleural effusion]], [[ascites]], [[chest]] [[tenderness]], [[abdomen]] [[tenderness]], [[bone]] [[tenderness]], [[arthritis]], [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]]. [[Medical laboratory|Laboratory]] [[Test|tests]] for angioimmunoblastic T-cell lymphoma include [[complete blood count|complete blood count (CBC)]], polyclonal hypergammaglobulinemia, [[FISH]], [[blood]] chemistry studies, [[flow cytometry]], [[immunohistochemistry]], and [[immunophenotyping]]. [[Abdomen|Abdominal]] [[Computed tomography|CT]] scan may be helpful in the [[diagnosis]] of angioimmunoblastic T-cell lymphoma. Findings on [[Computed tomography|CT scan]] suggestive of angioimmunoblastic T-cell lymphoma include [[Lymphadenopathy|mediastinal lymphadenopathy]], [[Lymphadenopathy|inguinal lymphadenopathy]], [[Lymphadenopathy|aortal lymphadenopathy]], and [[Bilateral|bilaterally]] enlarged [[Kidney|kidneys]]. [[MRI]] may be helpful in the [[diagnosis]] of angioimmunoblastic T-cell lymphoma. [[Lymph node]] or extranodal tissue [[biopsy]] is [[Diagnosis|diagnostic]] of angioimmunoblastic T-cell lymphoma. [[Abdomen|Abdominal]] [[ultrasound]] may be helpful in the [[diagnosis]] of angioimmunoblastic T-cell lymphoma. Findings on [[ultrasound]] of the [[abdomen]] suggestive of angioimmunoblastic T-cell lymphoma include [[hepatomegaly]] and [[splenomegaly]]. [[Positron emission tomography|Positron emission tomographic scan]] may be helpful in the [[diagnosis]] of angioimmunoblastic T-cell lymphoma. Findings on [[Positron emission tomography|positron emission tomographic scan]] suggestive of angioimmunoblastic T-cell lymphoma include [[lymphadenopathy]] and [[Bilateral|bilaterally]] enlarged [[Kidney|kidneys]]. Other [[Diagnosis|diagnostic]] studies for angioimmunoblastic T-cell lymphoma include [[laparoscopy]], [[laparotomy]], [[bone marrow aspiration]], and [[bone marrow biopsy]]. The predominant therapy for angioimmunoblastic T-cell lymphoma is [[chemotherapy]]. Adjunctive [[stem cell transplantation]] may be required in certain cases. | |||
==Pathophysiology== | ==Pathophysiology== | ||
Clonal [[T-cell receptor]] and [[immunoglobulin]] gene rearrangements are involved in the pathogenesis of angioimmunoblastic T-cell lymphoma. On gross pathology, aborization and proliferation of [[high endothelial venules]] are characteristic findings of angioimmunoblastic T-cell lymphoma. On microscopic histopathological analysis, [[CD4]]+ [[T-cells]], [[CD8]]+ [[T-cells]], and polyclonal [[plasma cells]] are characteristic findings of angioimmunoblastic T-cell lymphoma. | Clonal [[T-cell receptor]] and [[immunoglobulin]] [[gene]] rearrangements are involved in the [[pathogenesis]] of angioimmunoblastic T-cell lymphoma. On [[gross pathology]], aborization and proliferation of [[high endothelial venules]] are characteristic findings of angioimmunoblastic T-cell lymphoma. On [[microscopic]] [[Histopathology|histopathological]] [[analysis]], [[CD4]]+ [[T-cells]], [[CD8]]+ [[T-cells]], and polyclonal [[plasma cells]] are characteristic findings of angioimmunoblastic T-cell lymphoma. | ||
==Causes== | ==Causes== | ||
There are no established causes for angioimmunoblastic T-cell lymphoma. | There are no established causes for angioimmunoblastic T-cell lymphoma. | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
Angioimmunoblastic T-cell lymphoma must be differentiated from other diseases such as | Angioimmunoblastic T-cell lymphoma must be differentiated from other diseases such as peripheral T-cell lymphoma-not otherwise specified, [[classical Hodgkin's lymphoma]], [[diffuse large B cell lymphoma]], and [[nodal marginal zone lymphoma]]. | ||
==Epidemiology and demographics== | ==Epidemiology and demographics== | ||
Angioimmunoblastic T-cell lymphoma commonly affects middle-aged or elderly patients. Angioimmunoblastic T-cell lymphoma affects males and females equally. | Angioimmunoblastic T-cell lymphoma commonly affects middle-aged or elderly patients. Angioimmunoblastic T-cell lymphoma affects males and females equally. | ||
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There are no established risk factors for angioimmunoblastic T-cell lymphoma. | There are no established risk factors for angioimmunoblastic T-cell lymphoma. | ||
==Screening== | ==Screening== | ||
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for angioimmunoblastic T-cell lymphoma. | According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for angioimmunoblastic T-cell lymphoma. | ||
==Complications== | ==Complications== | ||
Common complications of angioimmunoblastic T-cell lymphoma include [[rheumatoid arthritis]], [[autoimmune hemolytic anemia]], [[vasculitis]], and autoimmune thyroid disease. | Common [[Complication (medicine)|complications]] of angioimmunoblastic T-cell lymphoma include [[rheumatoid arthritis]], [[autoimmune hemolytic anemia]], [[vasculitis]], and [[Autoimmunity|autoimmune]] [[thyroid]] disease. | ||
==Diagnosis== | ==Diagnosis== | ||
===Staging=== | ===Staging=== | ||
According to the Lugano classification, there are four stages of angioimmunoblastic T-cell lymphoma based on the number of nodes and extranodal involvement. | According to the Lugano [[classification]], there are four stages of angioimmunoblastic T-cell lymphoma based on the number of [[Lymph node|nodes]] and extranodal involvement. | ||
===Symptoms=== | ===Symptoms=== | ||
The most common symptoms of angioimmunoblastic T-cell lymphoma include [[fever]], [[weight loss]], skin rash, [[night sweats]], [[edema]], chest pain, [[abdominal pain]], [[bone pain]], [[fatigue]], [[dark urine]], [[shortness of breath]], [[chronic pain]], swelling of joints, and painless swelling of the neck, axilla, groin, thorax, and abdomen. | The most common [[Symptom|symptoms]] of angioimmunoblastic T-cell lymphoma include [[fever]], [[weight loss]], [[Rash|skin rash]], [[night sweats]], [[edema]], [[chest pain]], [[abdominal pain]], [[bone pain]], [[fatigue]], [[dark urine]], [[shortness of breath]], [[chronic pain]], [[Edema|swelling]] of [[Joint|joints]], and painless swelling of the [[neck]], [[axilla]], [[groin]], [[Chest|thorax]], and [[abdomen]]. | ||
===Physical Examination=== | ===Physical Examination=== | ||
Common physical examination findings of angioimmunoblastic T-cell lymphoma include [[fever]], [[rash]], [[ulcer]], [[splenomegaly]], [[hepatomegaly]], [[pleural effusion]], [[ascites]], chest tenderness, abdomen tenderness, bone tenderness, [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]]. | Common [[physical examination]] findings of angioimmunoblastic T-cell lymphoma include [[fever]], [[rash]], [[ulcer]], [[splenomegaly]], [[hepatomegaly]], [[pleural effusion]], [[ascites]], [[chest]] tenderness, [[abdomen]] [[tenderness]], [[bone]] [[tenderness]], [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]]. | ||
===Laboratory Tests=== | ===Laboratory Tests=== | ||
Laboratory tests for angioimmunoblastic T-cell lymphoma include [[complete blood count | [[Medical laboratory|Laboratory]] [[Test|tests]] for angioimmunoblastic T-cell lymphoma include [[complete blood count|complete blood count (CBC)]], polyclonal hypergammaglobulinemia, [[FISH]], [[blood]] chemistry studies, [[flow cytometry]], [[immunohistochemistry]], and [[immunophenotyping]]. | ||
===CT=== | ===CT=== | ||
Abdominal CT scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on CT scan suggestive of angioimmunoblastic T-cell lymphoma include [[Lymphadenopathy|mediastinal lymphadenopathy]], [[Lymphadenopathy|inguinal lymphadenopathy]], [[Lymphadenopathy|aortal lymphadenopathy]], and bilaterally enlarged kidneys. | [[Abdomen|Abdominal]] [[Computed tomography|CT scan]] may be helpful in the [[diagnosis]] of angioimmunoblastic T-cell lymphoma. Findings on [[Computed tomography|CT scan]] suggestive of angioimmunoblastic T-cell lymphoma include [[Lymphadenopathy|mediastinal lymphadenopathy]], [[Lymphadenopathy|inguinal lymphadenopathy]], [[Lymphadenopathy|aortal lymphadenopathy]], and [[Bilateral|bilaterally]] enlarged [[Kidney|kidneys]]. | ||
===MRI=== | ===MRI=== | ||
[[MRI]] | [[MRI]] may be helpful in the [[diagnosis]] of angioimmunoblastic T-cell lymphoma. | ||
===Biopsy=== | ===Biopsy=== | ||
Lymph node or extranodal tissue biopsy is diagnostic of angioimmunoblastic T-cell lymphoma. | [[Lymph node]] or extranodal tissue [[biopsy]] is [[Diagnosis|diagnostic]] of angioimmunoblastic T-cell lymphoma. | ||
===Ultrasound=== | ===Ultrasound=== | ||
Abdominal ultrasound may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on ultrasound abdomen suggestive of angioimmunoblastic T-cell lymphoma include [[hepatomegaly]] and [[splenomegaly]]. | [[Abdomen|Abdominal]] [[ultrasound]] may be helpful in the [[diagnosis]] of angioimmunoblastic T-cell lymphoma. Findings on [[ultrasound]] of the [[abdomen]] suggestive of angioimmunoblastic T-cell lymphoma include [[hepatomegaly]] and [[splenomegaly]]. | ||
===Other Imaging Studies=== | ===Other Imaging Studies=== | ||
Positron emission tomographic | [[Positron emission tomography|Positron emission tomographic scan]] may be helpful in the [[diagnosis]] of angioimmunoblastic T-cell lymphoma. Findings on [[Positron emission tomography|positron emission tomographic scan]] suggestive of angioimmunoblastic T-cell lymphoma include [[lymphadenopathy]] and [[Bilateral|bilaterally]] enlarged [[Kidney|kidneys]]. | ||
===Other Diagnostic Studies=== | ===Other Diagnostic Studies=== | ||
Other diagnostic studies for angioimmunoblastic T-cell lymphoma include [[laparoscopy]], [[laparotomy]], [[bone marrow aspiration]], and [[bone marrow biopsy]]. | Other [[Diagnosis|diagnostic]] studies for angioimmunoblastic T-cell lymphoma include [[laparoscopy]], [[laparotomy]], [[bone marrow aspiration]], and [[bone marrow biopsy]]. | ||
==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
Latest revision as of 21:16, 15 April 2019
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Angioimmunoblastic T-cell lymphoma Microchapters |
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Differentiating Angioimmunoblastic T-cell lymphoma from other Diseases |
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Angioimmunoblastic T-cell lymphoma overview On the Web |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2] Sowminya Arikapudi, M.B,B.S. [3]
Overview
Angioimmunoblastic T-cell lymphoma (AILT) is a mature T-cell lymphoma characterized by a polymorphous lymph node infiltrate showing a marked increase in follicular dendritic cells (FDCs) and high endothelial venules (HEVs). It is also known as immunoblastic lymphadenopathy (Lukes-Collins Classification) and AILD-type (lymphogranulomatosis X) T-cell lymphoma (Kiel Classification). Clonal T-cell receptor and immunoglobulin gene rearrangements are involved in the pathogenesis of angioimmunoblastic T-cell lymphoma. On gross pathology, aborization and proliferation of high endothelial venules are characteristic findings of angioimmunoblastic T-cell lymphoma. On microscopic histopathological analysis, CD4+ T-cells, CD8+ T-cells, and polyclonal plasma cells are characteristic findings of angioimmunoblastic T-cell lymphoma. There are no established causes for angioimmunoblastic T-cell lymphoma. Angioimmunoblastic T-cell lymphoma must be differentiated from other diseases such as peripheral T-cell lymphoma-not otherwise specified, classical Hodgkin's lymphoma, diffuse large B cell lymphoma, and nodal marginal zone lymphoma. Angioimmunoblastic T-cell lymphoma commonly affects middle-aged or elderly individuals. Angioimmunoblastic T-cell lymphoma affects men and women equally. There are no established risk factors for angioimmunoblastic T-cell lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for angioimmunoblastic T-cell lymphoma. Common complications of angioimmunoblastic T-cell lymphoma include rheumatoid arthritis, autoimmune hemolytic anemia, vasculitis, and autoimmune thyroid disease. According to the Lugano classification, there are four stages of angioimmunoblastic T-cell lymphoma based on the number of nodes and extranodal involvement. The most common symptoms of angioimmunoblastic T-cell lymphoma include fever, weight loss, skin rash, night sweats, edema, chest pain, abdominal pain, bone pain, fatigue, dark urine, shortness of breath, chronic pain, swelling of joints, and painless swelling of the neck, axilla, groin, thorax, and abdomen. Common physical examination findings of angioimmunoblastic T-cell lymphoma include fever, rash, ulcer, splenomegaly, hepatomegaly, pleural effusion, ascites, chest tenderness, abdomen tenderness, bone tenderness, arthritis, peripheral lymphadenopathy, and central lymphadenopathy. Laboratory tests for angioimmunoblastic T-cell lymphoma include complete blood count (CBC), polyclonal hypergammaglobulinemia, FISH, blood chemistry studies, flow cytometry, immunohistochemistry, and immunophenotyping. Abdominal CT scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on CT scan suggestive of angioimmunoblastic T-cell lymphoma include mediastinal lymphadenopathy, inguinal lymphadenopathy, aortal lymphadenopathy, and bilaterally enlarged kidneys. MRI may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Lymph node or extranodal tissue biopsy is diagnostic of angioimmunoblastic T-cell lymphoma. Abdominal ultrasound may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on ultrasound of the abdomen suggestive of angioimmunoblastic T-cell lymphoma include hepatomegaly and splenomegaly. Positron emission tomographic scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on positron emission tomographic scan suggestive of angioimmunoblastic T-cell lymphoma include lymphadenopathy and bilaterally enlarged kidneys. Other diagnostic studies for angioimmunoblastic T-cell lymphoma include laparoscopy, laparotomy, bone marrow aspiration, and bone marrow biopsy. The predominant therapy for angioimmunoblastic T-cell lymphoma is chemotherapy. Adjunctive stem cell transplantation may be required in certain cases.
Pathophysiology
Clonal T-cell receptor and immunoglobulin gene rearrangements are involved in the pathogenesis of angioimmunoblastic T-cell lymphoma. On gross pathology, aborization and proliferation of high endothelial venules are characteristic findings of angioimmunoblastic T-cell lymphoma. On microscopic histopathological analysis, CD4+ T-cells, CD8+ T-cells, and polyclonal plasma cells are characteristic findings of angioimmunoblastic T-cell lymphoma.
Causes
There are no established causes for angioimmunoblastic T-cell lymphoma.
Differential Diagnosis
Angioimmunoblastic T-cell lymphoma must be differentiated from other diseases such as peripheral T-cell lymphoma-not otherwise specified, classical Hodgkin's lymphoma, diffuse large B cell lymphoma, and nodal marginal zone lymphoma.
Epidemiology and demographics
Angioimmunoblastic T-cell lymphoma commonly affects middle-aged or elderly patients. Angioimmunoblastic T-cell lymphoma affects males and females equally.
Risk Factors
There are no established risk factors for angioimmunoblastic T-cell lymphoma.
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for angioimmunoblastic T-cell lymphoma.
Complications
Common complications of angioimmunoblastic T-cell lymphoma include rheumatoid arthritis, autoimmune hemolytic anemia, vasculitis, and autoimmune thyroid disease.
Diagnosis
Staging
According to the Lugano classification, there are four stages of angioimmunoblastic T-cell lymphoma based on the number of nodes and extranodal involvement.
Symptoms
The most common symptoms of angioimmunoblastic T-cell lymphoma include fever, weight loss, skin rash, night sweats, edema, chest pain, abdominal pain, bone pain, fatigue, dark urine, shortness of breath, chronic pain, swelling of joints, and painless swelling of the neck, axilla, groin, thorax, and abdomen.
Physical Examination
Common physical examination findings of angioimmunoblastic T-cell lymphoma include fever, rash, ulcer, splenomegaly, hepatomegaly, pleural effusion, ascites, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.
Laboratory Tests
Laboratory tests for angioimmunoblastic T-cell lymphoma include complete blood count (CBC), polyclonal hypergammaglobulinemia, FISH, blood chemistry studies, flow cytometry, immunohistochemistry, and immunophenotyping.
CT
Abdominal CT scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on CT scan suggestive of angioimmunoblastic T-cell lymphoma include mediastinal lymphadenopathy, inguinal lymphadenopathy, aortal lymphadenopathy, and bilaterally enlarged kidneys.
MRI
MRI may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma.
Biopsy
Lymph node or extranodal tissue biopsy is diagnostic of angioimmunoblastic T-cell lymphoma.
Ultrasound
Abdominal ultrasound may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on ultrasound of the abdomen suggestive of angioimmunoblastic T-cell lymphoma include hepatomegaly and splenomegaly.
Other Imaging Studies
Positron emission tomographic scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on positron emission tomographic scan suggestive of angioimmunoblastic T-cell lymphoma include lymphadenopathy and bilaterally enlarged kidneys.
Other Diagnostic Studies
Other diagnostic studies for angioimmunoblastic T-cell lymphoma include laparoscopy, laparotomy, bone marrow aspiration, and bone marrow biopsy.
Treatment
Medical Therapy
The predominant therapy for angioimmunoblastic T-cell lymphoma is chemotherapy. Adjunctive stem cell transplantation may be required.
References