Important note: Information in this article was accurate in 1991. The state of the art may have changed since the publication date.
ADULT T-CELL LEUKEMIA/LYMPHOMA
Retrovirus Biology and Human Disease. Gallo RC and Wong-Staal F, eds. Marcel Dekker, New York, p. 147-59, 1990.. Unique Identifier : AIDSLINE ICDB/91676222 Takatsuki K; Yamaguchi K; Hattori T; The Second Dept. of Internal Medicine, Kumamoto Univ. Medical; Sch., Kumamoto, Japan
Abstract:
Adult T-cell leukemia/lymphoma (ATL) was first recognized as a distinct clinical entity in Japan in the early 1970s. ATL is caused by human T-cell lymphotropic virus type I (HTLV-I) infection, and southwestern Japan is the most endemic area in the world. ATL is reviewed, including clinical features, classification, diagnosis, accompanying diseases, epidemiology, familial occurrence, pathophysiology, and treatment of ATL and prevention of HTLV-I infection. The authors studied 187 patients (pts) with ATL (113 men, 74 women, ages at onset 27-82 yr, av 55) in Kyushu, Japan. Clinical, hematologic, immunologic, cytogenetic, and virologic features were compared to those of pts from the Caribbean and Africa. These features and the clinical course, ATL subtype, frequency of hypercalcemia and opportunistic infections, cell morphology, phenotypic profile, and response to treatment were the same in both Japanese and non-Japanese groups. ATL pts are classified clinically into five subtypes: acute, chronic, smoldering, crisis, and lymphoma. Serum specimens from all pts with ATL have anti-HTLV-I antibodies. Immunologic studies of ATL cells have revealed phenotypic heterogeneity of T-cell subset markers, but most ATL cells have the CD4 antigen. HTLV-I provirus DNA was confirmed in peripheral blood mononuclear cells and/or lymph node cells of all pts with ATL and of some with T-cell malignant lymphomas. A frequent complication noted among those with smoldering ATL is cancer of other organs. Examination of pts with cancer in hospitals in Kumamoto Prefecture showed that, of 394 pts with malignancies, 15.5% who had not had blood transfusions were seropositive for HTLV-I antibody. These results suggest that HTLV-I infection may contribute to the risk of other malignancies. A study of 26 relatives of two pts with ATL suggested two modes of transmission of HTLV-I: parents to children and between spouses, especially from husband to wife. HTLV-I gene transcript or expression of HTLV-I-related antigens could not be detected in fresh ATL cells, but HTLV-I gene expression was detectable immediately after the cells were transferred into culture systems, suggesting that there are unknown mechanisms by which expression of HTLV-I-related antigens is suppressed in ATL cells. Pts with acute and lymphoma-type ATL should be treated with combination chemotherapy directed toward achieving a cure. However, pts with hypercalcemia, high lactate dehydrogenase levels, and an abnormal increase in WBC have a 50% survival time of less than 6 mo. Although vaccination against HTLV-I is possible, screening of blood donors and prevention of transmission via breast milk can be used to limit the spread outside endemic areas. (52 Refs)
Keywords: Adult Aged Aged, 80 and over Female Gene Expression Regulation, Leukemic/*PHYSIOLOGY Gene Expression Regulation, Viral/*PHYSIOLOGY Human HTLV-I/DRUG EFFECTS/*GENETICS Leukemia-Lymphoma, T-Cell, Acute, HTLV-I-Associated/DIAGNOSIS/ DRUG THERAPY/GENETICS/*MICROBIOLOGY Male Middle Age Pentostatin/THERAPEUTIC USE Transcription, Genetic/GENETICS Virus Replication/GENETICS MONOGRAPH
AEGiS presents published material, reprinted with permission and neither endorses nor opposes any material. All information contained on this website, including information relating to health conditions, products, and treatments, is for informational purposes only. It is often presented in summary or aggregate form. It is not meant to be a substitute for the advice provided by your own physician or other medical professionals. Always discuss treatment options with a doctor who specializes in treating HIV.
Important note: Information in this article was accurate in 1991. The state of the art may have changed since the publication date.
