Adult T-cell leukemia
| 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 |
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
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.
Pathophysiology
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.
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.
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.
Epidemiology and Demographics
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
- 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
Symptoms
Physical Examination
Skin
- Maculopapular rash
- Skin ulceration
- Leukemia cutis - leukemic infiltration of skin
Head
Abdomen
Laboratory Findings
- Complete blood count
- Hypercalcemia - hallmark of the disease
- Skin biopsy
- Bone marrow biopsy
- Peripheral smear
CT
- Used to determine the extent of the disease.
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
- 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.
- Epidemiologic Notes and Reports: Adult T-Cell Leukemia/Lymphoma Associated With Human T-Lymphotropic Virus Type I (HTLV-I) Infection -- North Carolina
- Genoveffa Franchini's NCI page: Human Retroviral Diseases: Pathogenesis and Prevention