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   MeshID        = D015460 |
   MeshID        = D015460 |
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}}
{{SI}}
{{Adult T-cell leukemia}}
{{CMG}}; {{AE}} {{RT}}
{{CMG}}; {{AE}} {{RT}}


{{SK}} T-cell leukaemia, adult
{{SK}} T-cell leukaemia, adult


==Overview==
==[[Adult T-cell leukemia overview|Overview]]==
Human T cell leukemia/lymphotropic virus type 1 ([[HTLV-1]]) is believed to be the cause of several diseases, including '''adult T cell leukemia/lymphoma''' ('''ATLL'''), a rare [[cancer]] of the immune system's own [[T-cell]]s.


==Pathophysiology==
==[[Adult T-cell leukemia historical perspective|Historical Perspective]]==
ATLL is usually a highly aggressive [[non-Hodgkin's lymphoma]] with no characteristic histologic appearance except for a diffuse pattern and a mature T-cell phenotype. Circulating [[lymphocyte]]s with an irregular nuclear contour (leukemic cells) are frequently seen. Several lines of evidence suggest that HTLV-1 causes ATLL. This evidence includes the frequent isolation of HTLV-1 from patients with this disease and the detection of HTLV-1 proviral genome in ATLL leukemic cells. ATLL is frequently accompanied by visceral involvement, [[hypercalcemia]], lytic bone lesions, and [[skin lesion]]s.


===Transmission===
==[[Adult T-cell leukemia classification|Classification]]==
Transmission of HTLV-1 is believed to occur from mother to child; by sexual contact; and through exposure to contaminated blood, either through [[blood transfusion]] or sharing of contaminated needles.


Infection with HTLV-1, like infection with other [[retroviruses]], probably occurs for life and can be inferred when antibody against HTLV-1 is detected in the [[blood plasma|serum]].
==[[Adult T-cell leukemia pathophysiology|Pathophysiology]]==


==Epidemiology and Demographics==
==[[Adult T-cell leukemia causes|Causes]]==
===Prevalence===
HTLV-1 infection in the United States appears to be rare. Although little serologic data exist, prevalence of infection is thought to be highest among blacks living in the Southeast. A prevalence rate of 30% has been found among black intravenous drug abusers in New Jersey, and a rate of 49% has been found in a similar group in New Orleans. It is possible that prevalence of infection is increasing in this risk group. Studies of HTLV-1 antibody indicate that the virus is endemic in southern Japan, in the Caribbean, and in Africa.
===Incidence===
ATLL is relatively uncommon among those infected with HTLV-1. The overall incidence of ATLL is estimated at about 66 per 100,000 adult HTLV-1 carriers per year.
===Race===
Those cases that have been reported have occurred mostly among persons from the Caribbean or blacks from the Southeast (National Institutes of Health, unpublished data). There appears to be a long latent period between HTLV-1 infection and the start of ATLL.


==Natural History, Complications and Prognosis==
==[[Adult T-cell leukemia differential diagnosis|Differentiating Adult T-cell leukemia from Other Diseases]]==
* Most patients die within one year of diagnosis.
* Possible complications from [[hypercalcemia]], which is a hallmark of the disease, include:
** Irregular heart rhythms
** Severe [[constipation]]


==Diagnosis==
==[[Adult T-cell leukemia epidemiology and demographics|Epidemiology and Demographics]]==
===Symptoms===
 
* [[Fatigue]]
==[[Adult T-cell leukemia risk factors|Risk Factors]]==
* [[Maculopapular rash]]
* [[Skin ulceration]]


===Physical Examination===
==[[Adult T-cell leukemia screening|Screening]]==
====Skin====
* Maculopapular rash
* Skin ulceration
* [[Leukemia cutis]] - leukemic infiltration of skin


====Head====
==[[Adult T-cell leukemia natural history|Natural History, Complications and Prognosis]]==
* [[Lymphadenopathy]]


====Abdomen====
==Diagnosis==
* [[Splenomegaly]]
[[Adult T-cell leukemia diagnostic criteria|Diagnostic Criteria]] | [[Adult T-cell leukemia staging|Staging]] | [[Adult T-cell leukemia history and symptoms|History and Symptoms]] | [[Adult T-cell leukemia physical examination|Physical Examination]] |  [[Adult T-cell leukemia laboratory findings|Laboratory Findings]] | [[Adult T-cell leukemia chest x ray|Chest X Ray]] | [[Adult T-cell leukemia CT|CT]] | [[Adult T-cell leukemia MRI|MRI]] | [[Adult T-cell leukemia echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Adult T-cell leukemia other imaging findings|Other Imaging Findings]] | [[Adult T-cell leukemia other diagnostic studies|Other Diagnostic Studies]]


===Laboratory Findings===
==Treatment==
* Complete blood count
[[Adult T-cell leukemia medical therapy|Medical Therapy]] | [[Adult T-cell leukemia surgery|Surgery]] | [[Adult T-cell leukemia primary prevention|Primary Prevention]] | [[Adult T-cell leukemia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Adult T-cell leukemia future or investigational therapies|Future or Investigational Therapies]]
* [[Hypercalcemia]] - hallmark of the disease
* Skin biopsy
* Bone marrow biopsy
* Peripheral smear


====CT====
==Case Studies==
* Used to determine the extent of the disease.
[[Adult T-cell leukemia case study one|Case #1]]


==Treatment==
The common regimens used to treat adult T-cell leukemia include
* [[CHOP]] regimen - [[cyclophosphamide]], [[doxorubicin]],[[vincristine]] and [[prednisone]]
* EPOCH regimen - [[etoposide]], [[vincristine]], [[doxorubicin]], [[cyclophosphamide]] and [[prednisone]]


== External links ==
== External links ==
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* Genoveffa Franchini's NCI page: [http://ccr.cancer.gov/staff/staff.asp?profileid=5587 Human Retroviral Diseases: Pathogenesis and Prevention]
* Genoveffa Franchini's NCI page: [http://ccr.cancer.gov/staff/staff.asp?profileid=5587 Human Retroviral Diseases: Pathogenesis and Prevention]
* [http://htlv.net International Retrovirology Association]
==References==
{{reflist|2}}


{{Hematological malignancy histology}}
{{Hematological malignancy histology}}
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[[Category:Types of cancer]]
[[Category:Types of cancer]]
[[Category:Virology]]
[[Category:Virology]]
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Revision as of 19:56, 18 September 2012

Adult T-cell leukemia
Adult T cell leukemia: Blood smear from a patient from the Kyushu region of Japan with HTLV-1-positive adult T-cell leukemia. The lymphocyte has a markedly lobulated, "flower" shaped nucleus. (Wright-Giemsa stain)
Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
ICD-10 C83-C88
ICD-9 204.0-208.9
ICD-O: Template:ICDO
DiseasesDB 29486
MeSH D015460

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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]

Synonyms and keywords: T-cell leukaemia, adult

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 Criteria | Staging | History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1


External links

  • Franchini G, Nicot C, Johnson JM (2003). "Seizing of T cells by human T-cell leukemia/lymphoma virus type 1". Adv. Cancer Res. 89: 69–132. PMID 14587871.
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