What is iatrogenic (immunosuppression-related) Kaposi sarcoma (KS)?

Updated: Apr 11, 2019
  • Author: Jessica Katz, MD, PhD, FACP; Chief Editor: Edwin Choy, MD, PhD  more...
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Answer

This entity can occur following solid-organ transplantation or in patients receiving immunosuppressive therapy for other indications. The incidence of Kaposi sarcoma is increased 100-fold in transplant patients [23, 24] However, individuals with congenital immunodeficient states are not at increased risk for developing Kaposi sarcoma. Iatrogenic Kaposi sarcoma is rare, [1] but is more common in patients at risk for the classic form of  the disease. [25]

The average time to development of Kaposi sarcoma following transplantation is 15-30 months. This subtype is usually aggressive and commonly presents as lymph node, mucosa, and visceral involvement. Disease regression is frequently seen with reduction or withdrawal of immunosuppressive therapy. [26] This is further evidence for the important role that immune suppression may play in the development of Kaposi sarcoma. Immune activation and suppression affect the natural history of HHV-8 in a very complex manner. [27]

One drug used for preventing transplant rejection, sirolimus (Rapamune), appears to have an anti–Kaposi sarcoma effect independent of its immunosuppressive action. [28, 29]  In a study of 15 kidney transplant patients with biopsy-proven Kaposi sarcoma who were switched from cyclosporine to sirolimus, all cutaneous lesions in all patients resolved. [30]

Sirolimus may have a therapeutic effect in Kaposi sarcoma due to its antiangiogenesis effect, with reduction of vascular endothelial growth factor (VEGF) and the Flk-1/KDR receptor on tumor cells, as well as its inhibition of the mammalian target of rapamycin (mTOR) pathway by blocking of Akt. Notably, mTor can activate various mediators of proliferation and affect control points of translation in the cell cycle. 


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