HIV/AIDS-Related Refractory Kaposi Sarcoma Causing Severe Leg Lymphedema.

A 24-year-old man from Guinea, presented with a 1-year history of swelling of his right leg. Physical examination revealed massive lymphedema along with formation of indurated and partly violaceous dermal plaques (Figure 1). Histological examination of a skin biopsy specimen confirmed Kaposi sarcoma (KS). Etiological investigation led to detection of HIV infection at the AIDS stage (CD4-positive T helper cells 60/µl, HIV viral load 115.000 copies/ml) and significant human-herpesvirus-8 (HHV-8) viremia [1, 2]. Combination antiretroviral therapy (cART) was initiated with raltegravir and emtricitabine/tenofovir. In addition, systemic chemotherapy with pegylated liposomal doxorubicin was administered due to advanced stage “T1” AIDS-related KS according to the AIDS Clinical Trials Group/Krown-Staging [3]. Pegylated liposomal doxorubicin (8 cycles per 16 weeks) proved inefficient (Figure 2A). Therefore, therapy was switched to paclitaxel (12 cycles per 24 weeks), which led to only limited clinical improvement (Figure 2B); despite that, recovery of the T helper cells counts up to 200/µl and maximal suppression of HIV and HHV-8 viremia were achieved after 42 cART weeks. According to current literature, KS usually regress within 24 weeks after initiation of cART [4]. Ultimately, the patient underwent therapy with vinorelbine (20 cycles per 48 weeks) and local irradiation (10 à 3 Gy), which resulted in significant physical improvement [5]. One year later, he remains in complete remission with a good quality of life despite residual right leg lymphedema (Figure 2C). This case represents a once-common clinical scenario that indeed has become increasingly rare with early antiviral treatment.

Figure 1.

Massive lymphedema of the right leg caused by Kaposi Sarcoma.

Figure 2.

A, Persistent lymphedema after therapy with pegylated liposomal doxorubicin. B, Slight improvement of leg lymphedema after therapy with paclitaxel. C, Complete remission of Kaposi Sarcoma along with only residual leg lymphedema 12 months after completion of therapy with vinorelbine and local irradiation.