Human immunodeficiency virus negative Kaposi sarcoma and lymphoproliferative disorders

The concomitant occurrence of more than one primary neoplasm in the same individual has led researchers to seek possible common etiopathogenetic factors. Kaposi sarcoma (KS) is a multicentric neoplasm of vascular origin and perhaps viral etiology. Four forms of KS are known: classic or Mediterranean, endemic or African, posttransplant, and epidemic or acquired immunodeficiency syndrome-associated KS. In its classic form KS mainly affects elderly people and often has a long and indolent course that occasionally allows other malignancies to appear. Previous studies of the possible association between human immunodeficiency virus (HIV) negative KS and lymphoproliferative disorders (LDs) have produced discordant results.

To verify a possibly significant association between HIV negative KS and LDs, data relating to 250 evaluable Italian patients with HIV negative KS were evaluated retrospectively.

Of the 250 KS patients, only 6 (2.4%) were found to have had an LD: 2 with Hodgkin lymphoma, 1 with non-Hodgkin lymphoma, 1 with cutaneous T-cell lymphoma, 1 with acute promyelocytic leukemia, and 1 with B-chronic lymphocytic leukemia.

No significant association was found between HIV negative KS and LDs in the patient population in the current study. The authors believe that age, LD, or therapy-related immunodepression played a role in the cases in which KS appeared after the LD by determining the passing to the lytic phase of the herpes-virus HHV8 already present in anatomic sites of latency/persistence.