Classical Kaposi’s Sarcoma in Caucasians in Africa – Experience at the Johannesburg Hospital (1978–1992)

Abstract

The data are scant concerning classical Kaposi’s sarcoma (CKS) among the Caucasian population of Africa. A description of the clinical features of 15 such patients, treated and followed up at the Johannesburg General Hospital over a 14-year period (1978–1992) is presented. All patients were negative for the human immunodeficiency virus. After a mean follow-up of 50 months (range: 7–168 months), 2 patients are alive with absent or minimal disease; 1 patient is alive with stable disease and 1 has active disease involving his extremities. Three patients were lost to follow-up but had minimal or no disease when last seen. Five patients died of unrelated causes but also had minimal or no disease at their last visit. One patient died of sepsis related to active KS. Only two patients died of progressive KS. No alterations in humoral or cellular immunity were demonstrated in 2 patients with persistent disease. Four (27%) patients developed lymphoproliferative disorders including non-Hodgkin’s lymphoma, Walden-ström’s macroglobulinemia, Hodgkin’s disease and Castleman’s disease (angio-follicular lymph node hyperplasia) preceding or following the diagnosis of CKS. These data confirm the indolent nature and good outcome of the classical form of Kaposi’s sarcoma among Caucasians in the southern African region. The marked association between CKS and lymphoproliferative disorders warrants a long-term awareness and continued monitoring for these complications.

Human immunodeficiency virus negative Kaposi sarcoma and lymphoproliferative disorders

“…A large number of reports of other associations followed3–10 but, although the well known article published by Safai et al in 1980 is among the most frequently cited in the literature because of the significance of its data (18 of 92 KS patients also had LDs), the results collected in various studies of large series of homogeneous patient populations with nonepidemic KS have not always been concordant (Table 1). 8, 11–21 All 250 patients with classic KS who have been treated at the Second Department of Dermatology in Milan since 1978 were included in this retrospective study designed to verify the presence of any associated LDs.…”

HHV‐8 subtypes in South Africa: Identification of a case suggesting a novel B variant

“…Since the start of the AIDS epidemic and the rapid spread of human immunodeficiency virus type 1 (HIV-1) in subSaharan Africa, differentiation between African endemic and epidemic Kaposi's sarcoma has become more difficult; presently the epidemic form is diagnosed predominantly in African patients [Matondo, 1995]. In South Africa, classic Kaposi's sarcoma is seen mostly in Jewish people of Eastern European (Ashkenazi) origin [Stein et al, 1994].…”

Classical Kaposi sarcoma

“…The course of CKS is usually indolent over many years and is generally not life threatening, as opposed to the aggressive course associated with the African or the acquired immunodeficiency syndrome (AIDS)‐related Kaposi sarcoma (KS) 2. The morphology is similar among all forms KS and is characterized by endothelial‐linked vascular channels, spindle‐shaped cells, and hemosiderin‐containing macrophages 3…”

“…2 The morphology is similar among all forms KS and is characterized by endothelial-linked vascular channels, spindle-shaped cells, and hemosiderin-containing macrophages. 3 The etiology of KS is unknown. However, several epidemiologic and environmental factors, as well as immunosuppression, play a role in the development and clinical course of the disease.…”