Adult T-cell leukemia

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Adult T-cell leukemia
Classification and external resources
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: M9827/3
DiseasesDB 29486
MeSH D015460

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Adult T-cell leukemia

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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-cells.

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 lymphocytes 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 lesions. Most patients die within one year of diagnosis.

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 serum.

Incidence/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.

ATLL is relatively uncommon among those infected with HTLV-1. The overall incidence of ATLL is estimated at about 1 per 1,500 adult HTLV-1 carriers per year. 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.

Transmission

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.

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.

de:Adulte T-Zell-Leukämie

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .

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