Adult T-cell leukemia overview: Difference between revisions

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{{Adult T-cell leukemia}}
{{Adult T-cell leukemia}}
{{CMG}} {{AE}} {{HL}}
{{CMG}}; {{AE}} {{HL}}; {{GRR}} {{Nat}}
==Overview==
==Overview==
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]].<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref> Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobated [[nucleus]] and agranular [[cytoplasm]]. Based on both the clinical presentation and lab values, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693  }} </ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370  }} </ref> The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. The natural history of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[cardiac arrhythmia]]s, [[opportunistic infection]]s , and [[bone fracture]]s. The prognosis varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor prognosis, where as chronic and smoldering subtypes have a good prognosis. The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell]] transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic [[stem cell]] transplant.<ref name="NCCN">Adult T-Cell Leukemia/Lymphoma. NCCN Guidelines Version 2 (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on January, 25 2016</ref>
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s, induced by an [[infection]] with [[Human T-lymphotropic virus|human T‐cell lymphotropic virus]] ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[Lymphocyte|lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]]. Based on both the clinical presentation and lab values, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant. The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. The natural history of adult T-cell leukemia varies between the different sub-types of the disease. Common complications of adult T-cell leukemia include [[cardiac arrhythmia]]s, [[opportunistic infection]]s, and [[bone fracture]]s. The prognosis varies between the sub-types of adult T-cell leukemia. Acute and lymphomatous sub-types have a poor prognosis; whereas chronic and smoldering sub-types have a good prognosis. The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell]] transplant, or a combination of zidovudine and interferon therapy. The first line [[Chemotherapy|chemotherapeutic]] regimens used for the initial management of adult T-cell leukemia include [[CHOP]], [[CHOEP regimen|CHOEP]], or [[DA-EPOCH regimen|Dose-adjusted EPOCH]]. Second line [[Chemotherapy|chemotherapeutic]] agents might be [[DHAP regimen|DHAP]], [[ESHAP regimen|ESHAP]], GDP, [[GemOx-R regimen|GemOx]], or [[ICE-R regimen|ICE]]. Surgery is not the first-line treatment option for patients with adult T-cell leukemia. [[Splenectomy]] is usually reserved for certain cases of adult T-cell leukemia. No preventive vaccine against [[Human T-lymphotropic virus|HTLV]]-1 is currently available.


==Historical Perspective==
==Historical Perspective==
Adult T-cell leukemia was first discovered by Dr. K. Takatsuki, a Japanese physician, in 1977. The association between [[HTLV]] infection and adult T-cell leukemia was made in 1981.<ref name="pmid20425378">{{cite journal| author=Mahieux R, Gessain A| title=Adult T-cell leukemia/lymphoma and HTLV-1. | journal=Curr Hematol Malig Rep | year= 2007 | volume= 2 | issue= 4 | pages= 257-64 | pmid=20425378 | doi=10.1007/s11899-007-0035-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20425378  }} </ref><ref name="pmid22507774">{{cite journal| author=Tsukasaki K| title=Adult T-cell leukemia-lymphoma. | journal=Hematology | year= 2012 | volume= 17 Suppl 1 | issue=  | pages= S32-5 | pmid=22507774 | doi=10.1179/102453312X13336169155330 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22507774  }} </ref>
Adult T-cell leukemia was first discovered in 1977 by Dr. K. Takatsuki, a Japanese physician. The association between [[HTLV]] infection and adult T-cell leukemia was made in 1981.


==Classification==
==Classification==
Based on both the clinical presentation and lab values, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smouldering variant, or a [[lymphoma]] variant.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693  }} </ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370  }} </ref>
Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an [[acute]] variant, [[chronic]] variant, smoldering variant, or an adult T-cell [[lymphoma]] variant.


==Pathophysiology==
==Pathophysiology==
Adult T‐cell leukemia arises from post‐thymic [[lymphocyte]]s, which are normally involved in the process of [[immune system|cell-mediated immune response]].<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="patho">Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015</ref><ref name="patho2">Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015</ref> Development of adult T-cell leukemia is the result of multiple [[genetic mutation]]s induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[nodule]]s, [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobated [[nucleus]] and agranular [[cytoplasm]].
Adult T‐cell leukemia arises from post‐thymic [[Lymphocyte|lymphocytes]], which are normally involved in the process of [[Immune system|cell-mediated immune response]]. Development of adult T-cell leukemia is the result of multiple [[Genetic mutation|genetic mutations]] induced by an [[infection]] with human T‐cell lymphotropic virus ([[HTLV]]). On gross pathology, skin [[Nodule|nodules]], [[maculopapular]] eruption, and [[erythema]] are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized [[lymphocytes]] with a polylobulated [[nucleus]] and agranular [[cytoplasm]].


==Causes==
==Causes==
Adult T-cell leukemia is caused by an infection with [[HTLV]]. Common [[genetic mutation]]s involved in the development of adult T-cell leukemia can be found [[Adult T-cell leukemia pathophysiology|'''here''']].<ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref>
Adult T-cell leukemia is caused by an infection with [[HTLV]]. Common [[Genetic mutation|genetic mutations]] involved in the development of adult T-cell leukemia can be found [[Adult T-cell leukemia pathophysiology|here]].


==Differentiating Adult T-cell leukemia from other Diseases==
==Differentiating Adult T-cell leukemia from other Diseases==
Adult T-cell leukemia must be differentiated from other diseases that cause [[weight loss]], [[night sweats]], [[hepatosplenomegaly]], and palpable [[lymph node]]s, such as [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].<ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="H">Hoffbrand V, Moss P. Essential Haematology. John Wiley & Sons; 2011</ref>
Adult T-cell leukemia must be differentiated from other diseases that cause [[weight loss]], [[night sweats]], [[hepatosplenomegaly]], and palpable [[Lymph node|lymph nodes]], such as [[hairy cell leukaemia|hairy cell leukemia]], prolymphocytic leukemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].


==Epidemiology and Demographics==
==Epidemiology and Demographics==
The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. In southern Japan, the age-adjusted [[incidence]] rate of adult T-cell leukemia is approximately 6.6 per 100,000 individuals. The incidence of adult T-cell leukemia increases with age; the median age at diagnosis is 57 years. Males are more commonly affected with adult T-cell leukemia than females. The male to female ratio is approximately 1.4 to 1. Adult T-cell leukemia usually affects individuals of the African American, Latin American, and Asian race. Caucasian individuals are less likely to develop adult T-cell leukemia.<ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="pmid20425378">{{cite journal| author=Mahieux R, Gessain A| title=Adult T-cell leukemia/lymphoma and HTLV-1. | journal=Curr Hematol Malig Rep | year= 2007 | volume= 2 | issue= 4 | pages= 257-64 | pmid=20425378 | doi=10.1007/s11899-007-0035-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20425378  }} </ref><ref name="pmid25219595">{{cite journal| author=Satake M, Yamada Y, Atogami S, Yamaguchi K| title=The incidence of adult T-cell leukemia/lymphoma among human T-lymphotropic virus type 1 carriers in Japan. | journal=Leuk Lymphoma | year= 2015 | volume= 56 | issue= 6 | pages= 1806-12 | pmid=25219595 | doi=10.3109/10428194.2014.964700 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25219595  }} </ref><ref name="pmid22973265">{{cite journal| author=Iwanaga M, Watanabe T, Yamaguchi K| title=Adult T-cell leukemia: a review of epidemiological evidence. | journal=Front Microbiol | year= 2012 | volume= 3 | issue=  | pages= 322 | pmid=22973265 | doi=10.3389/fmicb.2012.00322 | pmc=PMC3437524 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22973265  }} </ref>
The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. In southern Japan, the age-adjusted [[incidence]] rate of adult T-cell leukemia is approximately 6.6 per 100,000 individuals. The incidence of adult T-cell leukemia increases with age, and the median age at diagnosis is 57 years. Males are more commonly affected with adult T-cell leukemia than females. The male to female ratio is approximately 1.4 to 1. Adult T-cell leukemia usually affects individuals of the African American, Latin American, and Asian race. Caucasian individuals are less likely to develop adult T-cell leukemia.


==Risk Factors==
==Risk Factors==
Common risk factors in the development of adult T-cell leukemia among [[HTLV]] carriers are vertical transmission of HTLV [[infection]] during [[infancy]], male sex, and specific [[human leukocyte antigen]]s such as [[HLA-A]] 26, [[HLA-B]] 4002, and HLA-B 4801.<ref name="pmid22973265">{{cite journal| author=Iwanaga M, Watanabe T, Yamaguchi K| title=Adult T-cell leukemia: a review of epidemiological evidence. | journal=Front Microbiol | year= 2012 | volume= 3 | issue=  | pages= 322 | pmid=22973265 | doi=10.3389/fmicb.2012.00322 | pmc=PMC3437524 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22973265  }} </ref>
Common [[risk factors]] in the development of adult T-cell leukemia among [[HTLV]] carriers are [[vertical transmission]] of HTLV [[infection]] during [[Childbirth|labor]], male sex, and specific [[Human leukocyte antigen|human leukocyte antigens]] such as [[HLA-A]] 26, [[HLA-B]] 4002, and [[HLA-B]] 4801.  


==Screening==
==Screening==
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for adult T-cell leukemia.<ref name="US">Recommendations. US Preventive Service (2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Adult+T-cell+leukemia+ Accessed on January, 24 2015</ref>
According to the the U.S. Preventive Service Task Force ([[United states preventive services task force recommendations scheme|USPSTF]]), there is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for adult T-cell leukemia.


==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==
The natural history of adult T-cell leukemia varies between the different subtypes of the disease. Common complications of adult T-cell leukemia include [[cardiac arrhythmia]]s, [[opportunistic infection]]s , and [[bone fracture]]s. The prognosis varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor prognosis, where as chronic and smouldering subtypes have a good prognosis.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693  }} </ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="pmid26361794">{{cite journal| author=Katsuya H, Ishitsuka K, Utsunomiya A, Hanada S, Eto T, Moriuchi Y et al.| title=Treatment and survival among 1594 patients with ATL. | journal=Blood | year= 2015 | volume= 126 | issue= 24 | pages= 2570-7 | pmid=26361794 | doi=10.1182/blood-2015-03-632489 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26361794  }} </ref><ref name="pmid20425378">{{cite journal| author=Mahieux R, Gessain A| title=Adult T-cell leukemia/lymphoma and HTLV-1. | journal=Curr Hematol Malig Rep | year= 2007 | volume= 2 | issue= 4 | pages= 257-64 | pmid=20425378 | doi=10.1007/s11899-007-0035-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20425378  }} </ref>
The [[Natural history of disease|natural history]] of adult T-cell leukemia varies between the different sub-types of the disease. Common complications of adult T-cell leukemia include [[Cardiac arrhythmia|cardiac arrhythmias]], [[Opportunistic infection|opportunistic infections]] , and [[Bone fracture|bone fractures]]. The [[prognosis]] varies between the sub-types of adult T-cell leukemia. Acute and lymphomatous sub-types have a poor [[prognosis]]; whereas chronic and smouldering sub-types have a good [[prognosis]].


==Diagnosis==
==Diagnosis==
===Diagnostic Study of Choice===
There is no single diagnostic study of choice for the diagnosis of adult T-cell leukemia. However, adult T-cell leukemia can be diagnosed based on clinical manifestation and laboratory findings confirming characteristic [[histopathology]] and [[Human T-lymphotropic virus|HTLV]]-1 infection.
===History and Symptoms===
===History and Symptoms===
Symptoms of adult T-cell leukemia include [[fatigue]], [[fever]], [[night sweat]]s, [[constipation]], and reccurrent [[infection]]s.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693  }} </ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="pmid20425378">{{cite journal| author=Mahieux R, Gessain A| title=Adult T-cell leukemia/lymphoma and HTLV-1. | journal=Curr Hematol Malig Rep | year= 2007 | volume= 2 | issue= 4 | pages= 257-64 | pmid=20425378 | doi=10.1007/s11899-007-0035-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20425378  }} </ref>
Symptoms of adult T-cell leukemia include [[fatigue]], [[fever]], [[night sweat]]s, [[constipation]], and recurrent [[infection]]s.


===Physical Examination===
===Physical Examination===
Patients with adult T-cell leukemia usually appear lethargic and fatigued. Physical examination of patients with adult T-cell leukemia is usually remarkable for [[maculopapular]] [[rash]], skin [[ulceration]], and [[splenomegaly]].<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693  }} </ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref>
Patients with adult T-cell leukemia usually appear lethargic and fatigued. Physical examination of patients with adult T-cell leukemia is usually remarkable for [[maculopapular]] [[rash]], skin [[ulceration]], and [[splenomegaly]].


===Laboratory Findings===
===Laboratory Findings===
Laboratory findings consistent with the diagnosis of adult T-cell leukemia include abnormal [[anemia]], [[thrombocytopenia]], and elevated [[lymphocyte]] count. [[Hypercalcemia]] is a key feature among patients with adult T-cell leukemia.<ref name="pmid18042693">Matutes E (2007) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=18042693 Adult T-cell leukaemia/lymphoma.] ''J Clin Pathol'' 60 (12):1373-7. [http://dx.doi.org/10.1136/jcp.2007.052456 DOI:10.1136/jcp.2007.052456] PMID: [http://pubmed.gov/18042693 18042693]</ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370  }} </ref><ref name="pmid20425378">{{cite journal| author=Mahieux R, Gessain A| title=Adult T-cell leukemia/lymphoma and HTLV-1. | journal=Curr Hematol Malig Rep | year= 2007 | volume= 2 | issue= 4 | pages= 257-64 | pmid=20425378 | doi=10.1007/s11899-007-0035-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20425378  }} </ref>
Laboratory findings consistent with the diagnosis of adult T-cell leukemia include abnormal [[anemia]], [[thrombocytopenia]], and elevated [[lymphocyte]] count. [[Hypercalcemia]] is a key feature among patients with adult T-cell leukemia.
 
===Electrocardiogram===
There are no ECG findings associated with adult T-cell leukemia. However, [[electrocardiogram]] might be helpful in detecting complications of adult T-cell leukemia such as [[Cardiac arrhythmia|cardiac arrhythmias]] due to [[hypercalcemia]]. To view the electrocardiogram findings in [[hypercalcemia]], click [[Hypercalcemia electrocardiogram|here]].
 
===X-ray===
There are no x-ray findings associated with adult T-cell leukemia. However, an x-ray may be helpful in the diagnosis of complications of adult T-cell leukemia, which include [[bone fractures]] and lytic lesions.
 
=== Echocardiography and Ultrasound ===
There are no [[echocardiography]] findings associated with adult T-cell leukemia. Findings on an [[ultrasound]] suggestive of adult T-cell leukemia include [[hepatomegaly]], [[splenomegaly]] and [[lymphadenopathy]].


===CT Scan===
===CT Scan===
[[Thoracic]] CT scan may be helpful in the diagnosis of adult T-cell leukemia.<ref name="pmid14975983">{{cite journal| author=Okada F, Ando Y, Kondo Y, Matsumoto S, Maeda T, Mori H| title=Thoracic CT findings of adult T-cell leukemia or lymphoma. | journal=AJR Am J Roentgenol | year= 2004 | volume= 182 | issue= 3 | pages= 761-7 | pmid=14975983 | doi=10.2214/ajr.182.3.1820761 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975983  }} </ref> Findings on CT scan suggestive of [[pulmonary]] infiltration by adult T-cell leukemia cells include thickening of the bronchovascular bundles, consolidation in the peripheral [[lung]] [[parenchyma]], and ground-glass attenuations.
[[Thoracic]] [[CT scan]] may be helpful in the diagnosis of adult T-cell leukemia. Findings on [[CT scan]] suggestive of [[pulmonary]] infiltration by adult T-cell leukemia cells include thickening of the bronchovascular bundles, consolidation in the peripheral [[lung]] [[parenchyma]], and ground-glass attenuations. Findings on abdominal [[CT scan]] suggestive of adult T-cell leukemia cells include [[hepatomegaly]] and [[splenomegaly]].
 
===MRI===
There are no MRI findings associated with adult T-cell leukemia.
 
=== Other Imaging Findings ===
There are no other imaging findings associated with adult T-cell leukemia.


===Other Diagnostic Studies===
===Other Diagnostic Studies===
Other diagnostic studies for adult T-cell leukemia include [[skin]] [[biopsy]], [[bone marrow]] biopsy, and [[fluorescent in situ hybridization]]'''.<ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693  }} </ref><ref name="cancer">Leukemia - Chronic T-Cell Lymphocytic: Diagnosis. Cancer.Net (2015) http://www.cancer.net/cancer-types/leukemia-chronic-t-cell-lymphocytic/diagnosis Accessed on January, 25 2016</ref>'''
Other diagnostic studies for adult T-cell leukemia include [[skin]] [[biopsy]], [[bone marrow]] biopsy, and [[fluorescent in situ hybridization|fluorescent in-situ hybridization]]'''.'''


==Medical Therapy==
==Treatment==
The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell]] transplant, or a combination of zidovudine and interferon therapy. While adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic stem cell transplant.<ref name="NCCN">Adult T-Cell Leukemia/Lymphoma. NCCN Guidelines Version 2 (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on January, 25 2016</ref>
===Medical Therapy===
The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, [[skin]] directed therapies, or a combination of [[zidovudine]] and [[interferon]] therapy. Acute adult T-cell leukemia patients are usually managed by either [[chemotherapy]], supportive care, allogeneic [[stem cell transplant]], or a combination of [[zidovudine]] and [[interferon]] therapy. The first line [[Chemotherapy|chemotherapeutic]] regimens used for the initial management of adult T-cell leukemia include [[CHOP]], [[CHOEP regimen|CHOEP]], or [[DA-EPOCH regimen|Dose-adjusted EPOCH]]. Second line [[Chemotherapy|chemotherapeutic]] agents might be [[DHAP regimen|DHAP]], [[ESHAP regimen|ESHAP]], GDP, [[GemOx-R regimen|GemOx]], or [[ICE-R regimen|ICE]].  


==Surgery==
===Interventions===
Surgery is not the first-line treatment option for patients with adult T-cell leukemia. [[Splenectomy]] is usually reserved for certain cases of adult T-cell leukemia.<ref name="cancer">Leukemia - Chronic T-Cell Lymphocytic: Diagnosis. Cancer.Net (2015) http://www.cancer.net/cancer-types/leukemia-chronic-t-cell-lymphocytic/treatment-options Accessed on January, 25 2016</ref>
The mainstay of treatment for Adult T-cell leukemia is medical therapy. There are no recommended therapeutic interventions for the management of Adult T-cell leukemia.
 
===Surgery===
Surgery is not the first-line treatment option for patients with adult T-cell leukemia. [[Splenectomy]] is usually reserved for certain cases of adult T-cell leukemia.
 
=== Primary Prevention ===
Primary prevention of adult-T cell leukemia is aimed at preventing the vertical and person-person transmission of [[Human T-lymphotropic virus|HTLV]] virus. No preventive vaccine against [[Human T-lymphotropic virus|HTLV]]-1 is currently available.
 
=== Secondary Prevention ===
There are no established measures for the secondary prevention of Adult T-cell leukemia.


==References==
==References==
{{reflist|2}}
{{Reflist|2}}
 
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[[Category:Disease]]
[[Category:Medicine]]
[[Category:Pathology]]
[[Category:Hematology]]
[[Category:Hematology]]
[[Category:Immunology]]
[[Category:Infectious disease]]
[[Category:Oncology]]
[[Category:Up-To-Date]]

Latest revision as of 20:19, 29 July 2020

Adult T-cell leukemia Microchapters

Home

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Adult T-cell leukemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-Ray Findings

Echocardiography and Ultrasound

CT scan

MRI Findings

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Interventions

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]; Grammar Reviewer: Natalie Harpenau, B.S.[3]

Overview

Adult T‐cell leukemia arises from post‐thymic lymphocytes, which are normally involved in the process of cell-mediated immune response. Development of adult T-cell leukemia is the result of multiple genetic mutations, induced by an infection with human T‐cell lymphotropic virus (HTLV). On gross pathology, skin nodules, maculopapular eruption, and erythema are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobulated nucleus and agranular cytoplasm. Based on both the clinical presentation and lab values, adult T-cell leukemia may be classified into either an acute variant, chronic variant, smoldering variant, or an adult T-cell lymphoma variant. The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. The natural history of adult T-cell leukemia varies between the different sub-types of the disease. Common complications of adult T-cell leukemia include cardiac arrhythmias, opportunistic infections, and bone fractures. The prognosis varies between the sub-types of adult T-cell leukemia. Acute and lymphomatous sub-types have a poor prognosis; whereas chronic and smoldering sub-types have a good prognosis. The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of zidovudine and interferon therapy. Acute adult T-cell leukemia patients are usually managed by either chemotherapy, supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy. The first line chemotherapeutic regimens used for the initial management of adult T-cell leukemia include CHOP, CHOEP, or Dose-adjusted EPOCH. Second line chemotherapeutic agents might be DHAP, ESHAP, GDP, GemOx, or ICE. Surgery is not the first-line treatment option for patients with adult T-cell leukemia. Splenectomy is usually reserved for certain cases of adult T-cell leukemia. No preventive vaccine against HTLV-1 is currently available.

Historical Perspective

Adult T-cell leukemia was first discovered in 1977 by Dr. K. Takatsuki, a Japanese physician. The association between HTLV infection and adult T-cell leukemia was made in 1981.

Classification

Based on both the clinical presentation and laboratory findings, adult T-cell leukemia may be classified into either an acute variant, chronic variant, smoldering variant, or an adult T-cell lymphoma variant.

Pathophysiology

Adult T‐cell leukemia arises from post‐thymic lymphocytes, which are normally involved in the process of cell-mediated immune response. Development of adult T-cell leukemia is the result of multiple genetic mutations induced by an infection with human T‐cell lymphotropic virus (HTLV). On gross pathology, skin nodules, maculopapular eruption, and erythema are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobulated nucleus and agranular cytoplasm.

Causes

Adult T-cell leukemia is caused by an infection with HTLV. Common genetic mutations involved in the development of adult T-cell leukemia can be found here.

Differentiating Adult T-cell leukemia from other Diseases

Adult T-cell leukemia must be differentiated from other diseases that cause weight loss, night sweats, hepatosplenomegaly, and palpable lymph nodes, such as hairy cell leukemia, prolymphocytic leukemia, follicular lymphoma, and mantle cell lymphoma.

Epidemiology and Demographics

The majority of adult T-cell leukemia cases are reported in Japan, the Caribbean, South America, and Africa. In southern Japan, the age-adjusted incidence rate of adult T-cell leukemia is approximately 6.6 per 100,000 individuals. The incidence of adult T-cell leukemia increases with age, and the median age at diagnosis is 57 years. Males are more commonly affected with adult T-cell leukemia than females. The male to female ratio is approximately 1.4 to 1. Adult T-cell leukemia usually affects individuals of the African American, Latin American, and Asian race. Caucasian individuals are less likely to develop adult T-cell leukemia.

Risk Factors

Common risk factors in the development of adult T-cell leukemia among HTLV carriers are vertical transmission of HTLV infection during labor, male sex, and specific human leukocyte antigens such as HLA-A 26, HLA-B 4002, and HLA-B 4801.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for adult T-cell leukemia.

Natural History, Complications and Prognosis

The natural history of adult T-cell leukemia varies between the different sub-types of the disease. Common complications of adult T-cell leukemia include cardiac arrhythmias, opportunistic infections , and bone fractures. The prognosis varies between the sub-types of adult T-cell leukemia. Acute and lymphomatous sub-types have a poor prognosis; whereas chronic and smouldering sub-types have a good prognosis.

Diagnosis

Diagnostic Study of Choice

There is no single diagnostic study of choice for the diagnosis of adult T-cell leukemia. However, adult T-cell leukemia can be diagnosed based on clinical manifestation and laboratory findings confirming characteristic histopathology and HTLV-1 infection.

History and Symptoms

Symptoms of adult T-cell leukemia include fatigue, fever, night sweats, constipation, and recurrent infections.

Physical Examination

Patients with adult T-cell leukemia usually appear lethargic and fatigued. Physical examination of patients with adult T-cell leukemia is usually remarkable for maculopapular rash, skin ulceration, and splenomegaly.

Laboratory Findings

Laboratory findings consistent with the diagnosis of adult T-cell leukemia include abnormal anemia, thrombocytopenia, and elevated lymphocyte count. Hypercalcemia is a key feature among patients with adult T-cell leukemia.

Electrocardiogram

There are no ECG findings associated with adult T-cell leukemia. However, electrocardiogram might be helpful in detecting complications of adult T-cell leukemia such as cardiac arrhythmias due to hypercalcemia. To view the electrocardiogram findings in hypercalcemia, click here.

X-ray

There are no x-ray findings associated with adult T-cell leukemia. However, an x-ray may be helpful in the diagnosis of complications of adult T-cell leukemia, which include bone fractures and lytic lesions.

Echocardiography and Ultrasound

There are no echocardiography findings associated with adult T-cell leukemia. Findings on an ultrasound suggestive of adult T-cell leukemia include hepatomegaly, splenomegaly and lymphadenopathy.

CT Scan

Thoracic CT scan may be helpful in the diagnosis of adult T-cell leukemia. Findings on CT scan suggestive of pulmonary infiltration by adult T-cell leukemia cells include thickening of the bronchovascular bundles, consolidation in the peripheral lung parenchyma, and ground-glass attenuations. Findings on abdominal CT scan suggestive of adult T-cell leukemia cells include hepatomegaly and splenomegaly.

MRI

There are no MRI findings associated with adult T-cell leukemia.

Other Imaging Findings

There are no other imaging findings associated with adult T-cell leukemia.

Other Diagnostic Studies

Other diagnostic studies for adult T-cell leukemia include skin biopsy, bone marrow biopsy, and fluorescent in-situ hybridization.

Treatment

Medical Therapy

The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease. Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of zidovudine and interferon therapy. Acute adult T-cell leukemia patients are usually managed by either chemotherapy, supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy. The first line chemotherapeutic regimens used for the initial management of adult T-cell leukemia include CHOP, CHOEP, or Dose-adjusted EPOCH. Second line chemotherapeutic agents might be DHAP, ESHAP, GDP, GemOx, or ICE.

Interventions

The mainstay of treatment for Adult T-cell leukemia is medical therapy. There are no recommended therapeutic interventions for the management of Adult T-cell leukemia.

Surgery

Surgery is not the first-line treatment option for patients with adult T-cell leukemia. Splenectomy is usually reserved for certain cases of adult T-cell leukemia.

Primary Prevention

Primary prevention of adult-T cell leukemia is aimed at preventing the vertical and person-person transmission of HTLV virus. No preventive vaccine against HTLV-1 is currently available.

Secondary Prevention

There are no established measures for the secondary prevention of Adult T-cell leukemia.

References