Important note: Information in this article was accurate in 1988. The state of the art may have changed since the publication date.
ATYPICAL AGGRESSIVE KAPOSI'S SARCOMA IN AFRICA
Kaposi's Sarcoma: A Text and Atlas. Gottlieb GJ, Ackerman AB, eds. Philadelphia, Lea and Febiger, p. 151-70, 1988.. Unique Identifier : AIDSLINE ICDB/88647200 Bayley AC; Dept. of Surgery, Univ. of Zambia, Lusaka, Zambia
Abstract:
The epidemiologic, clinical, and pathologic characteristics of endemic Kaposi's sarcoma (KS) and atypical aggressive KS in Africa are reviewed, and the characteristics distinguishing these two disorders are discussed and tabulated. Clinical features of atypical aggressive KS are described in detail and histories from two cases are reported. Clinical features observed in atypical aggressive KS include lymphadenopathy, skin lesions, gastrointestinal lesions, weight loss, respiratory distress, CNS disease, pericardial effusions, and immune deficiency. Atypical aggressive KS has occurred with increasing frequency in both Uganda and Zimbabwe since 1983, but not in Kenya up through March 1986. Reports from Zaire and Rwanda, where acquired immune deficiency syndrome (AIDS) is common, suggest that atypical aggressive KS is rare or absent. There are similarities between lymphadenopathic KS in African children and atypical aggressive KS in adults. In both variations of the disease, symmetrical generalized lymph node enlargement is the most common sign, skin lesions are absent or few and on proximal sites, visceral lesions are common, responses to chemotherapy are uncertain, and the long-term prognosis is poor. However, when tested, none of four African children with KS were seropositive for human T-lymphotrophic virus type III (HTLV-III), whereas atypical aggressive KS in Africa is clearly related to HTLV-III and to immunosuppression. The atypical aggressive form also differs in important respects from AIDS-related KS as described in the Northern Hemisphere among homosexual men. AIDS-related KS tends to be overshadowed clinically by opportunistic infections, which are the most immediately life-threatening aspects of the disease. In contrast, with atypical aggressive KS, the sarcoma dominates the clinical picture in seropositive African patients. Atypical aggressive KS appears to not respond to chemotherapy as well as AIDS-related KS and appears to have a worse prognosis. With atypical aggressive KS, an initial response to actinomycin D and vincristine is observed, but it usually ceases after a few weeks, with the result that there is a high early mortality (61% at 6 mo post diagnosis in 1983). In contrast, death within a year from the time of onset of endemic KS is rare. (22 Refs)
Keywords: Acquired Immunodeficiency Syndrome/PATHOLOGY Africa Antineoplastic Agents, Combined/THERAPEUTIC USE Human Lymph Nodes/PATHOLOGY Neoplasms, Multiple Primary/PATHOLOGY Sarcoma, Kaposi's/DRUG THERAPY/*PATHOLOGY Skin/PATHOLOGY Skin Neoplasms/DRUG THERAPY/*PATHOLOGY MONOGRAPH
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