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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) and systemic involvement.<ref name="who1"/>  It is also known as '''immunoblastic lymphadenopathy''' (Lukes-Collins Classification) and '''AILD-type (lymphogranulomatosis X) T-cell lymphoma''' (Kiel Classification)<ref name="who1">[http://www.iarc.fr/WHO-BlueBooks/BBwebsite/bb3.html] Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): '''World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues.''' IARC Press: Lyon 2001 </ref>. Clonal [[T-cell receptor]] and [[immunoglobulin]] gene rearrangements are involved in the pathogenesis of angioimmunoblastic T-cell lymphoma. On gross pathology, polymorphous infiltrate, residual follicles, and blast-like [[B-cells]] 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. 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. 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]], [[joint pain]], chest pain, [[abdominal pain]], [[bone pain]] and painless swelling in 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]], [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|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 [[Lymphadenopathy|mediastinal lymphadenopathy]], [[Lymphadenopathy|inguinal lymphadenopathy]], [[Lymphadenopathy|aortal lymphadenopathy]], and bilaterally enlarged kidneys. [[MRI]] scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Lymph node or extranodal tissue biopsy is diagnostic of angioimmunoblastic T-cell lymphoma. Abdomen 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]]. Positron emission tomographic [[CT]] scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on positron emission tomographic [[CT]] 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.  
'''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) and systemic involvement.<ref name="who1"/>  It is also known as '''immunoblastic lymphadenopathy''' (Lukes-Collins Classification) and '''AILD-type (lymphogranulomatosis X) T-cell lymphoma''' (Kiel Classification)<ref name="who1">[http://www.iarc.fr/WHO-BlueBooks/BBwebsite/bb3.html] Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): '''World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues.''' IARC Press: Lyon 2001 </ref>. Clonal [[T-cell receptor]] and [[immunoglobulin]] gene rearrangements are involved in the pathogenesis of angioimmunoblastic T-cell lymphoma. On gross pathology, polymorphous infiltrate, residual follicles, and blast-like [[B-cells]] 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. 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. 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]], [[joint pain]], chest pain, [[abdominal pain]], [[bone pain]], [[fatigue]], [[pale skin color]], [[dark urine]], [[shortness of breath]], [[chronic pain]], swelling of joints and painless swelling in 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]], [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|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 [[Lymphadenopathy|mediastinal lymphadenopathy]], [[Lymphadenopathy|inguinal lymphadenopathy]], [[Lymphadenopathy|aortal lymphadenopathy]], and bilaterally enlarged kidneys. [[MRI]] scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Lymph node or extranodal tissue biopsy is diagnostic of angioimmunoblastic T-cell lymphoma. Abdomen 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]]. Positron emission tomographic [[CT]] scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on positron emission tomographic [[CT]] 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.  


==Pathophysiology==
==Pathophysiology==
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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 nodes and extranodal involvement.
===Symptoms===
===Symptoms===
The most common symptoms of angioimmunoblastic T-cell lymphoma include [[fever]], [[weight loss]], skin rash, [[night sweats]],  [[edema]], [[joint pain]], chest pain, [[abdominal pain]], [[bone pain]] and painless swelling in the neck, axilla, groin, thorax, and abdomen.
The most common symptoms of angioimmunoblastic T-cell lymphoma include [[fever]], [[weight loss]], skin rash, [[night sweats]],  [[edema]], [[joint pain]], chest pain, [[abdominal pain]], [[bone pain]], [[fatigue]], [[pale skin color]], [[dark urine]], [[shortness of breath]], [[chronic pain]], swelling of joints and painless swelling in the neck, axilla, groin, 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, [[arthritis]], [[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, [[arthritis]], [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].

Revision as of 20:42, 21 December 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editor(s)-in-Chief: Raviteja Guddeti, 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) and systemic involvement.[1] It is also known as immunoblastic lymphadenopathy (Lukes-Collins Classification) and AILD-type (lymphogranulomatosis X) T-cell lymphoma (Kiel Classification)[1]. Clonal T-cell receptor and immunoglobulin gene rearrangements are involved in the pathogenesis of angioimmunoblastic T-cell lymphoma. On gross pathology, polymorphous infiltrate, residual follicles, and blast-like B-cells 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. 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. 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, joint pain, chest pain, abdominal pain, bone pain, fatigue, pale skin color, dark urine, shortness of breath, chronic pain, swelling of joints and painless swelling in 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 scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Lymph node or extranodal tissue biopsy is diagnostic of angioimmunoblastic T-cell lymphoma. Abdomen 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. Positron emission tomographic CT scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on positron emission tomographic CT 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.

Pathophysiology

Clonal T-cell receptor and immunoglobulin gene rearrangements are involved in the pathogenesis of angioimmunoblastic T-cell lymphoma. On gross pathology, polymorphous infiltrate, residual follicles, and blast-like B-cells 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. Angioimmunoblastic T-cell lymphoma affects men and women 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.

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, joint pain, chest pain, abdominal pain, bone pain, fatigue, pale skin color, dark urine, shortness of breath, chronic pain, swelling of joints and painless swelling in 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, arthritis, 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 scan 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

Abdomen 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.

Other Imaging Studies

Positron emission tomographic CT scan may be helpful in the diagnosis of angioimmunoblastic T-cell lymphoma. Findings on positron emission tomographic CT 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

  1. 1.0 1.1 [1] Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001


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