Background
- A tumour associated with HHV-8
- Closely associated with advanced HIV, but may also present as classic, endemic, or transplant-related KS
ACTG Staging
- Based on extent of tumour (T), immune status (I), and severity of systemic illness (S)
| Criterion | Lower Risk (0) | Higher risk (1) |
|---|---|---|
| Tumour (T) | Confined to skin and/or lymph nodes and/or minimal oral disease (non-nodular KS confined to palate) | Tumor-associated edema or ulceration; extensive oral KS; gastrointestinal KS; or KS in other non-nodal viscera |
| Immune status (I) | CD4 cell count >200/µL | CD4 cell count <200/µL |
| Systemic illness (S) | No history of OI or thrush; no "B" symptoms; and Karnofsky performance status >70 | History of OI or thrush; "B" symptoms present; Karnofsky performance status <70; or other HIV-related illness (eg, neurologic disease, lymphoma) |
- However, staging only distinguishes between good risk (T0I0S0) and poor risk (literally all others), used for predicting mortality in the pre-ART era
- The 3-year survival rate of patients post-ART with T1S1 is about 50%, whereas for T0S0, T1S0, and T0S1 was all 80-90%; immune status does not appear to be predictive[1]
Clinical Manifestations
- Non-tender, hyperpigmented skin lesions
- May be macular or nodular
- Oral lesions in about a third
- May involve lymphatics, causing severe edema
- May involve the viscera, which may be asymptomatic or cause dyspnea (lungs), hematochezia or melena (GI tract), or other signs and symptoms
IRIS
- Treatment of HIV-associated KS may cause IRIS, either associated with new lesions or with worsening of existing lesions
- May be "unmasking" (first presentation) or paradoxical worsening of pre-existing lesions
- Risk factors for IRIS include T1 tumour stage, pre-treatment HIV viral load > 5 logs, detectable HHV-8 viremia, and initiation of ART without concurrent chemotherapy
Management
- Treatment goals are symptom alleviation, prevention of disease progression, and shrinkage of tumour to alleviate edema, organ compromise, and psychological stress
HIV Patients
- Combination antiretroviral therapy is the mainstay of treatment for all patients with HIV
- Disease may worsen for 3 to 6 weeks following initiation of ART, due to immune reconstitution inflammatory syndrome
- Try to decrease or stop any corticosteroids, if possible, since it appears to worsen KS
Transplant Patients
Local Treatments
- Intralesional vinblastine 0.2 to 0.3 mg/mL solution with a volume of 0.1 mL per 0.5 cm2 of lesion
- May be repeated at 3 to 4 weeks
- Radiation therapy
- Topical alitretinoin
Systemic Chemotherapy
- Used in cases of advanced or rapidly-progressive disease
- Indications include:
- Symptomatic visceral involvement
- Widespread skin involvement (eg, more than 25 lesions)
- Extensive cutaneous KS that is unresponsive to local treatment
- Extensive edema
- Immune reconstitution inflammatory syndrome
- Progression of KS on ART alone
- Options include pegylated liposomal doxorubicin or liposomal daunorubicin, paclitaxel, bleomycin, vinblastine, vincristine, or etoposide
- First-line: liposomal doxorubicin 20 mg/m2 every three weeks
- Second-line: paclitaxel
Direct Antivirals
- In vitro activity of ganciclovir, foscarnet, and cidofovir has not translated into clinical efficacy
- Not recommended
IRIS
- Supportive care
- Chemotherapy
- Avoid steroids
Prognosis
Prognostic Index
- Predicts survival following the development of HAART[2]
Criteria
| Criterion | Score |
|---|---|
| KS as first AIDS-defining illness | -3 |
| Age ≥50 years | +2 |
| CD4 count | -1 for every 100 cells |
| S1 stage | 3 |
Interpretation
| Score | 6 months | 1 year | 2 years | 5 years |
|---|---|---|---|---|
| 0 | 99.8% | 99.3% | 99.0% | 98.4% |
| 5 | 98.7% | 96.7% | 94.6% | 91.8% |
| 10 | 93.3% | 83.4% | 74.1% | 63.1% |
| 15 | 69.2% | 37.8% | 19.9% | 8.4% |
Further Reading
- Human Herpesvirus-8 Disease. In: Guidelines for the Prevention and Treatment of Opportunistic Infections in Adults and Adolescents with HIV. NIH, CDC, HIVMA, and IDSA. Available at [1]
- ↑ Nasti G, Talamini R, Antinori A, Martellotta F, Jacchetti G, Chiodo F, Ballardini G, Stoppini L, Di Perri G, Mena M, Tavio M, Vaccher E, D'Arminio Monforte A, Tirelli U; AIDS Clinical Trial Group Staging System in the Haart Era--the Italian Cooperative Group on AIDS and Tumors and the Italian Cohort of Patients Naive from Antiretrovirals. AIDS-related Kaposi's Sarcoma: evaluation of potential new prognostic factors and assessment of the AIDS Clinical Trial Group Staging System in the Haart Era--the Italian Cooperative Group on AIDS and Tumors and the Italian Cohort of Patients Naive From Antiretrovirals. J Clin Oncol. 2003 Aug 1;21(15):2876-82. doi: 10.1200/JCO.2003.10.162. PMID: 12885804.
- ↑ Stebbing J, Sanitt A, Nelson M, Powles T, Gazzard B, Bower M. A prognostic index for AIDS-associated Kaposi's sarcoma in the era of highly active antiretroviral therapy. Lancet. 2006 May 6;367(9521):1495-502. doi: 10.1016/S0140-6736(06)68649-2. PMID: 16679162.
