Adult T-cell leukemia: Difference between revisions

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{{Infobox_Disease |
{{Adult T-cell leukemia}}
  Name          = {{PAGENAME}} |
{{CMG}}; {{AE}} {{HL}}{{AIDA}}; {{GRR}} {{Nat}}
  Image          = Adult T cell leukemia.jpg|
  Caption        = 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) <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>|
  DiseasesDB    = 29486 |
  ICD10          = {{ICD10|C|83||c|81}}-{{ICD10|C|88||c|81}} |
  ICD9          = {{ICD9|204.0}}-{{ICD9|208.9}} |
  ICDO          = {{ICDO|9827|3}} |
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
  MeshID        = D015460 |
}}
{{SI}}
{{CMG}}; {{AE}} {{RT}}


{{SK}} T-cell leukaemia, adult
{{SK}} T cell leukemia; T cell leukemias; T cell leukaemia; T cell leukaemias; Adult T cell leukemia; Adult T cell leukemias; Acute adult T-cell leukemia; Chronic adult T-cell leukemia; Smouldering adult T-cell leukemia; Adult T-cell lymphoma


==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]]
* [[Maculopapular rash]]
* [[Skin ulceration]]


===Physical Examination===
==[[Adult T-cell leukemia risk factors|Risk Factors]]==
====Skin====
* Maculopapular rash
* Skin ulceration
* [[Leukemia cutis]] - leukemic infiltration of skin


====Head====
==[[Adult T-cell leukemia screening|Screening]]==
* [[Lymphadenopathy]]


====Abdomen====
==[[T-cell leukemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
* [[Splenomegaly]]


===Laboratory Findings===
==Diagnosis==
* Complete blood count
[[Adult T-cell leukemia diagnostic study of choice|Diagnostic Study of Choice]] | [[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 electrocardiogram|Electrocardiogram]] | [[Adult T-cell leukemia x ray|X-Ray Findings]] | [[Adult T-cell leukemia echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Adult T-cell leukemia CT|CT scan]] | [[Adult T-cell leukemia MRI|MRI Findings]] | [[Adult T-cell leukemia other imaging findings|Other Imaging Findings]] | [[Adult T-cell leukemia other diagnostic studies|Other Diagnostic Studies]]
* [[Hypercalcemia]] - hallmark of the disease
* Skin biopsy
* Bone marrow biopsy
* Peripheral smear
 
====CT====
* Used to determine the extent of the disease.


==Treatment==
==Treatment==
The common regimens used to treat adult T-cell leukemia include
[[Adult T-cell leukemia medical therapy|Medical Therapy]] | [[Adult T-cell leukemia interventions|Interventions]] | [[Adult T-cell leukemia surgery|Surgery]] | [[Adult T-cell leukemia primary prevention|Primary Prevention]] | [[Adult T-cell leukemia secondary prevention|Secondary 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]]
* [[CHOP]] regimen - [[cyclophosphamide]], [[doxorubicin]],[[vincristine]] and [[prednisone]]
* EPOCH regimen - [[etoposide]], [[vincristine]], [[doxorubicin]], [[cyclophosphamide]] and [[prednisone]]


== External links ==
==Case Studies==
* {{cite journal |author=Franchini G, Nicot C, Johnson JM |title=Seizing of T cells by human T-cell leukemia/lymphoma virus type 1 |journal=Adv. Cancer Res. |volume=89 |issue= |pages=69-132 |year=2003 |pmid=14587871 |doi=}}
[[Adult T-cell leukemia case study one|Case #1]]


* Epidemiologic Notes and Reports: [http://www.cdc.gov/mmwr/preview/mmwrhtml/00001013.htm Adult T-Cell Leukemia/Lymphoma Associated With Human T-Lymphotropic Virus Type I (HTLV-I) Infection -- North Carolina]
[[Category:Medicine]]
 
[[Category:Hematology]]
* Genoveffa Franchini's NCI page: [http://ccr.cancer.gov/staff/staff.asp?profileid=5587 Human Retroviral Diseases: Pathogenesis and Prevention]
[[Category:Immunology]]
 
[[Category:Infectious disease]]
* [http://htlv.net International Retrovirology Association]
 
==References==
{{reflist|2}}
 
{{Hematological malignancy histology}}
 
[[Category:Disease]]
[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Hematology]]
[[Category:Up-To-Date]]
[[Category:Types of cancer]]
[[Category:Virology]]
 
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Latest revision as of 17:21, 25 July 2020

Adult T-cell leukemia Microchapters

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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

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Electrocardiogram

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CT scan

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Case #1

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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]Aida Javanbakht, M.D.; Grammar Reviewer: Natalie Harpenau, B.S.[3]

Synonyms and keywords: T cell leukemia; T cell leukemias; T cell leukaemia; T cell leukaemias; Adult T cell leukemia; Adult T cell leukemias; Acute adult T-cell leukemia; Chronic adult T-cell leukemia; Smouldering adult T-cell leukemia; Adult T-cell lymphoma

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

Case Studies

Case #1

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