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) 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>. | '''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>. | ||
AILT was originally thought to be a premalignant condition, termed angioimmunoblastic lymphadenopathy, and this atypical reactive lymphadenopathy carried a risk for transformation into a lymphoma. Currently, it is postulated that the originating cell for this disease is a mature (post-thymic) CD4+ [[T-cell]] that arises ''de novo'', although some researchers argue that there is a premalignant subtype of this disease.<ref name="fri1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=2642571&query_hl=22&itool=pubmed_ExternalLink] | |||
Frizzera G, Kaneko Y, Sakurai M. "Angioimmunoblastic lymphadenopathy and related disorders: a retrospective look in search of definitions." '''Leukemia'''. 1989 Jan;3(1):1-5. PMID: 2642571</ref><ref name="smi1"> | |||
[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10666395&query_hl=26&itool=pubmed_ExternalLink] | |||
Smith JL, Hodges E, Quin CT, McCarthy KP, Wright DH. "Frequent T and B cell oligoclones in histologically and immunophenotypically characterized angioimmunoblastic lymphadenopathy." '''Am J Pathol'''. 2000 Feb;156(2):661-9. PMID: 10666395</ref> The [[Epstein Barr virus]] ([[EBV]]) is observed in the majority of cases, and the virus has been found in the reactive B-cells that comprise part of the polymorphous infiltrate of this disease<ref name="wei1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=1373088&query_hl=15&itool=pubmed_ExternalLink] | |||
Weiss LM, Jaffe ES, Liu XF, Chen YY, Shibata D, Medeiros LJ. "Detection and localization of Epstein-Barr viral genomes in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma." '''Blood'''. 1992 Apr 1;79(7):1789-95. PMID: 1373088</ref> and in the neoplastic T-cells.<ref name="ana1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=1327284&query_hl=19&itool=pubmed_ExternalLink] | |||
Anagnostopoulos I, Hummel M, Finn T, Tiemann M, Korbjuhn P, Dimmler C, Gatter K, Dallenbach F, Parwaresch MR, Stein H. "Heterogeneous Epstein-Barr virus infection patterns in peripheral T-cell lymphoma of angioimmunoblastic lymphadenopathy type."'''Blood'''. 1992 Oct 1;80(7):1804-12. PMID: 1327284</ref> [[Immunodeficiency]] is also seen with this disease, but it is thought to be a sequela to the condition and not a predisposing factor. | |||
==Diagnosis== | ==Diagnosis== | ||
===Staging=== | ===Staging=== | ||
Revision as of 15:24, 27 November 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [6]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [7]
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].
AILT was originally thought to be a premalignant condition, termed angioimmunoblastic lymphadenopathy, and this atypical reactive lymphadenopathy carried a risk for transformation into a lymphoma. Currently, it is postulated that the originating cell for this disease is a mature (post-thymic) CD4+ T-cell that arises de novo, although some researchers argue that there is a premalignant subtype of this disease.[2][3] The Epstein Barr virus (EBV) is observed in the majority of cases, and the virus has been found in the reactive B-cells that comprise part of the polymorphous infiltrate of this disease[4] and in the neoplastic T-cells.[5] Immunodeficiency is also seen with this disease, but it is thought to be a sequela to the condition and not a predisposing factor.
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 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
- ↑ 1.0 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
- ↑ [2] Frizzera G, Kaneko Y, Sakurai M. "Angioimmunoblastic lymphadenopathy and related disorders: a retrospective look in search of definitions." Leukemia. 1989 Jan;3(1):1-5. PMID: 2642571
- ↑ [3] Smith JL, Hodges E, Quin CT, McCarthy KP, Wright DH. "Frequent T and B cell oligoclones in histologically and immunophenotypically characterized angioimmunoblastic lymphadenopathy." Am J Pathol. 2000 Feb;156(2):661-9. PMID: 10666395
- ↑ [4] Weiss LM, Jaffe ES, Liu XF, Chen YY, Shibata D, Medeiros LJ. "Detection and localization of Epstein-Barr viral genomes in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma." Blood. 1992 Apr 1;79(7):1789-95. PMID: 1373088
- ↑ [5] Anagnostopoulos I, Hummel M, Finn T, Tiemann M, Korbjuhn P, Dimmler C, Gatter K, Dallenbach F, Parwaresch MR, Stein H. "Heterogeneous Epstein-Barr virus infection patterns in peripheral T-cell lymphoma of angioimmunoblastic lymphadenopathy type."Blood. 1992 Oct 1;80(7):1804-12. PMID: 1327284