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| __NOTOC__
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| {{Angioimmunoblastic T-cell lymphoma}}
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| {{CMG}}; {{AE}} {{RT}}
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| ==Overview==
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| '''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>.
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| ==Pathophysiology==
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| ===Genetics===
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| Clonal [[T-cell receptor]] gene rearrangements are detected in 75% of cases<ref name="fell1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=2849301&query_hl=34&itool=pubmed_ExternalLink]
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| Feller AC, Griesser H, Schilling CV, Wacker HH, Dallenbach F, Bartels H, Kuse R, Mak TW, Lennert K. "Clonal gene rearrangement patterns correlate with immunophenotype and clinical parameters in patients with angioimmunoblastic lymphadenopathy." '''Am J Pathol'''. 1988 Dec;133(3):549-56. PMID: 2849301</ref>, and [[immunoglobin]] gene rearrangements are seen in 10% of cases, and these cases are believed to be due to expanded EBV-driven [[B-cell]] populations.<ref name="lip1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=3805286&query_hl=36&itool=pubmed_ExternalLink]
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| Lipford EH, Smith HR, Pittaluga S, Jaffe ES, Steinberg AD, Cossman J. "Clonality of angioimmunoblastic lymphadenopathy and implications for its evolution to malignant lymphoma." '''J Clin Invest'''. 1987 Feb;79(2):637-42. PMID: 3805286</ref> Similarly, [[EBV]]-related sequences can be detected most cases, usually in [[B-cells]] but occasionally in [[T-cells]].<ref name="wei1"/><ref name="ana1"/>. [[Trisomy]] 3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in cases.<ref name="kane1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=3261178&query_hl=40&itool=pubmed_ExternalLink]
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| Kaneko Y, Maseki N, Sakurai M, Takayama S, Nanba K, Kikuchi M, Frizzera G. "Characteristic karyotypic pattern in T-cell lymphoproliferative disorders with reactive "angioimmunoblastic lymphadenopathy with dysproteinemia-type" features." '''Blood'''. 1988 Aug;72(2):413-21. PMID: 3261178</ref><ref name="sch1">
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| [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=7919378&query_hl=42&itool=pubmed_ExternalLink]
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| Schlegelberger B, Zhang Y, Weber-Matthiesen K, Grote W. "Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics." '''Blood'''. 1994 Oct 15;84(8):2640-8. PMID: 7919378</ref>
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| ===Gross Pathology===
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| The normal architecture of a lymph node is partially effaced by a polymorphous infiltrate and residual follicles are commonly seen. The polymorphous infiltrate consists of lymphocytes of moderate size with pale/clear cytoplasm and smaller reactive [[lymphocytes]], [[eosinophils]], [[histiocytes]], [[plasma cells]], and [[follicular dendritic cells]]. In addition, blast-like [[B-cells]] are occasionally seen. A classic morphological finding is the aborization and proliferation of [[high endothelial venules]].<ref name="who1"/> Hyperplastic [[germinal centers]] and [[Reed-Sternberg cells]] can also be seen.<ref name="quin1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10524524&query_hl=30&itool=pubmed_ExternalLink]
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| Quintanilla-Martinez L, Fend F, Moguel LR, Spilove L, Beaty MW, Kingma DW, Raffeld M, Jaffe ES. "Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection." '''Am J Surg Pathol'''. 1999 Oct;23(10):1233-40. PMID: 10524524</ref><ref name="ree1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9630171&query_hl=32&itool=pubmed_ExternalLink]
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| Ree HJ, Kadin ME, Kikuchi M, Ko YH, Go JH, Suzumiya J, Kim DS. "Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplastic germinal centers." '''Am J Surg Pathol'''. 1998 Jun;22(6):643-55. PMID: 9630171</ref>
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| ===Microscopic Pathology===
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| AILT typically has the phenotype of a mixture of [[CD4]]+ and [[CD8]]+ [[T-cells]], with a CD4:CD8 ratio greater than unity. Polyclonal [[plasma cells]] and [[CD21]]+ [[follicular dendritic cells]] are also seen.<ref name="who1"/> Due to the systemic nature of this disease, neoplastic cells can be found in [[lymph nodes]], [[liver]], [[spleen]], [[skin]], and [[bone marrow]].
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| ==Causes==
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| 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]
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| 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">
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| [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10666395&query_hl=26&itool=pubmed_ExternalLink]
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| 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]
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| 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]
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| 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.
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| ==Overview==
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| AILT comprises 15-20% of peripheral [[T-cell lymphomas]] and 1-2% of all [[non-Hodgkin lymphomas]].<ref name="anon1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9166827&query_hl=8&itool=pubmed_ExternalLink]
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| Anon. "A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project." '''Blood'''. 1997 Jun 1;89(11):3909-18. PMID: 9166827</ref>
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| ==Age==
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| The typical patient with angioimmunoblastic T-cell lymphoma (AILT) is either middle-aged or elderly.
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| ==Gender==
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| No gender preference for this disease has been observed.
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| ==Symptoms==
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| Patients with this disease usually present at an advanced stage and show systemic involvement. The clinical findings typically include
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| * A pruritic skin [[rash]]
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| * [[Edema]]
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| * [[Ascites]]
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| * [[Pleural effusions]]
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| * [[Arthritis]].<ref name="sie1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8664186&query_hl=11&itool=pubmed_ExternalLink]
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| Siegert W, Nerl C, Agthe A, Engelhard M, Brittinger G, Tiemann M, Lennert K, Huhn D. "Angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma: prognostic impact of clinical observations and laboratory findings at presentation. The Kiel Lymphoma Study Group." '''Ann Oncol'''. 1995 Sep;6(7):659-64. PMID: 8664186</ref><ref name="jaf1">
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| [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8664181&query_hl=13&itool=pubmed_ExternalLink]
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| Jaffe ES. "Angioimmunoblastic T-cell lymphoma: new insights, but the clinical challenge remains." Ann Oncol. 1995 Sep;6(7):631-2. PMID: 8664181</ref>
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| ==Laboratory Findings==
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| The classical laboratory finding is polyclonal [[hypergammaglobulinemia]]
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| Other [[immunoglobulin]] derrangements are also seen, including
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| * [[Hemolytic anemia]] with [[cold agglutinins]]
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| * [[Circulating immune complexes]]
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| * [[Anti-smooth muscle antibodies]]
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| * Positive [[rheumatoid factor]]
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| ==Treatment==
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| ==References==
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| {{reflist|2}}
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| {{reflist|2}}
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| {{reflist|2}}
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| [[Category:Hematology]]
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| [[Category:Types of cancer]]
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| [[Category:Oncology]]
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| [[Category:Disease]]
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| [[Category:Needs content]]
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