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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2013  |  Volume : 24  |  Issue : 6  |  Page : 1199-1202
Kaposi's sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient

1 Department of Nephrology, La Rabta Hospital, Tunis, Tunisia
2 Department of Dermatology, La Rabta Hospital, Tunis, Tunisia
3 Department of Anatomo-Pathology, La Rabta Hospital, Tunis, Tunisia
4 Laboratory of Renal Pathology, LR001SP, Tunis, Tunisia

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Date of Web Publication13-Nov-2013


The association between Kaposi's sarcoma (KS) and human herpes virus eight (HHV-8) infection is rarely reported in hemodialysis (HD) patients. We report here the rare association of KS, HHV-8 and hepatitis C virus (HCV) infection as well as syphilis in a HD patient. We report the case of a 72-year-old woman who presented with microscopic polyangiitis with alveolar hemorrhage and pauci-immune necrosing and crescentic glomerulonephritis as well as renal failure requiring HD. Biological tests showed positive HCV and syphilis tests. The patient was treated by HD and intravenous pulse, followed by oral corticosteroids and six cyclophosphamide monthly pulses with remission of the alveolar hemorrhage, but without renal functional recovery as the patient remained HD dependent. Five months after the first treatment administration, she developed extensive purpuric lesions on her lower limbs, abdomen face and neck. A skin biopsy showed KS. The HHV-8 test was positive, with positive polymerase chain reaction-HHV8 in the serum and skin. After immunosuppression withdrawal, the KS skin lesions regressed rapidly without relapse after 12 months of follow-up, but alveolar hemorrhage relapsed after 16 months of follow-up. Our case showed that the immunosuppressed state related to multiple factors such as aging, vasculitis, HHV-8, HCV, syphilis, immunosuppressive therapy and HD may all have contributed to the development of KS in our patient.

How to cite this article:
Fatma LB, Rais L, Mebazza A, Azzouz H, Beji S, Krid M, Smaoui W, Maiz HB, Zouaghi K, Zitouna M, Osmane AB, Moussa FB. Kaposi's sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient. Saudi J Kidney Dis Transpl 2013;24:1199-202

How to cite this URL:
Fatma LB, Rais L, Mebazza A, Azzouz H, Beji S, Krid M, Smaoui W, Maiz HB, Zouaghi K, Zitouna M, Osmane AB, Moussa FB. Kaposi's sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient. Saudi J Kidney Dis Transpl [serial online] 2013 [cited 2023 Feb 4];24:1199-202. Available from: https://www.sjkdt.org/text.asp?2013/24/6/1199/121285

   Introduction Top

Kaposi's sarcoma (KS) was first described by Moritz von Kaposi in 1872 as a low-grade mesenchymal tumor involving the blood and lymphatic vessels. The mucocutaneous sites are primarily affected, typically the skin of the lower extremities, face, trunk, genitalia and the oropharyngeal mucosa; other organs like the digestive and pulmonary tracts are involved in the disseminated form of the disease. [1],[2]

KS is reported to be associated with human herpes virus eight (HHV-8). HHV-8 was discovered in 1994 in the biopsy of KS in a patient with acquired immunodeficiency syndrome (AIDS). Its prevalence ranges from 3.81 to 46.02%. [3] Since then, it has been identified in all variants of KS. [4] The incidence of iatrogenic KS, a cancer linked with HHV-8 following organ transplantation, is 500-times higher than that in the general population. The incidence of KS in the transplant population ranges from 2 to 3.5%. [5] In the Middle East, KS is the most common malignancy reported in kidney transplant recipients. [6] Differences in the prevalence of HHV-8 and KS have been described, depending on the study population and their geographic origin. In a cross-sectional study, serum samples were collected and evaluated for the presence and titers of HHV-8-specific antibodies, and it was suggested that a longitudinal evaluation of the antibody titers in patients with chronic kidney disease be undertaken to confirm their predictive value in the development of KS. [7]

In an immunocompromised host, the primary infection with HHV-8 presents with fever, hepatosplenomegaly, lymphoid hyperplasia, pancytopenia and liver dysfunction. Occasionally, rapid-onset KS develops in association with apparent primary HHV-8 infection. [5] Iatrogenic KS develops in patients undergoing immunosuppressive treatment and is considered to be induced by activation of latent HHV-8. In most cases, the first manifestation of KS develops after 1 year from when the drug was first administered. [8]

Finally, the simultaneous presence of two viruses such as HHV8 and hepatitis C virus (HCV) can create a synergy for the onset of KS. [8]

KS is related to HHV-8 infection and it is rarely reported in hemodialysis (HD) human immunodeficiency virus (HIV)-negative patients. We report here the rare association of KS, HHV-8 and HCV infection as well as syphilis in a HD patient.

   Case Report Top

A 72-year- old HIV-negative woman presented with weakness, microscopic hematuria, oliguria and rapidly progressive renal failure. A kidney biopsy revealed pauci-immune-necrosing crescentic glomerulonephritis [Figure 1]. P-ANCA was positive with anti-myeloperoxidase specificity. The diagnosis was microscopic polyangiitis with renal and pulmonary involvement including alveolar hemorrhage and a GOLD score at 150. Blood tests for HIV, syphilis and HCV/HBV were carried out and showed positive antibodies for syphilis (TPHA and VDRL) and HCV, with positive polymerase chain reaction (PCR) for HVC, but the blood test for HIV was negative. The patient was initiated on regular HD and intravenous corticosteroids, followed by oral prednisone (1 mg/kg/day) administered for a duration of 1 month then tapered by 5 mg every 15 days in addition to six-monthly cyclophosphamide intravenous pulses at dose 500 mg/1.73 m 2 . Unfortunately, the renal function failed to improve and the patient remained on chronic HD. Syphilis was treated with intramuscular benzathine benzylpenicillin injection repeated after 15 days.
Figure 1: Kidney biopsy (optical microscopic): Extracapillary glomerulonephritis.

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After 5 months from the initiation of treatment, the patient presented with multiple nodular purpuric lesions on her lower limbs, abdomen, face and neck [Figure 2], [Figure 3] and [Figure 4]. A skin biopsy diagnosed KS [Figure 5]. Blood tests were positive for HHV-8: IgG anti-HHV8 positive, titer 1/640. PCR amplification showed HHV-8 in the peripheral blood. Immunohistochemical stains for HHV-8 were positive in the skin sample. No lesions were detected in the gastrointestinal tract.
Figure 2: Kaposi lesions on the plant feet.

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Figure 3: Kaposi lesions on the lower left limb.

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Figure 4: Kaposi lesions on the face and neck.

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Figure 5: Skin biopsy of an infiltrated lesion (hematoxylin and eosin ×10): Double proliferation of fibroblastic cells and vascular structures increased spindle cells with vascular slits and vascular structures, with a predominance of endothelial cells. Extravasated erythrocytes and hemosiderin-laden macrophages. Some spindle cells may show nuclear pleomorphism.

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Withdrawal of immunosuppressive therapy was associated with complete regression of the KS lesions within 3 months without relapse. After a follow-up of 16 months, the patient remained on HD. Alveolar hemorrhage relapsed with iron-laden macrophages in the broncho - alveolar lavage and a GOLD score of 280. Anti-myloperoxidase P-ANCA was also positive.

   Discussion Top

Our case is unique if we consider the presence of multiple viral-related diseases, including HHV8, HCV and syphilis, besides immunosuppressive treatment in a patient with ANCA-associated vasculitis who is uremic and undergoing HD. In endemic countries, the seroprevalences of HCV and syphilis are reported to be 1.3% and 2.3%, respectively. [9] There is no report in the literature of an association between KS and syphilis. KS and kidney disease are frequently reported after kidney transplantation, and more rarely in native kidneys. Only one case of KS associated with microscopic polyangiitis with long-term corticosteroids has been reported, and our case is the second of such reports, with a difference in its association with HHV-8 and a shorter-term corticosteroid treatment. [10] In HD, reports of KS are rare, [11] and KS is seldom reported in immunocompetent patients. [4],[7]

A variety of therapeutic options exist and the choice of treatment depends on the clinical form and stage as well as the location of the lesions and their size; options include surgical excision, intralesional interferon alfa-2b, local or extended field radiotherapy and chemotherapy. [12]

Radiation therapy was also used with a complete response rate in 88% of the cases, with limited toxicity to the skin. [12] Paclitaxel has been approved as second-line therapy after anthracyclines in AIDS- KS patients according to the AIDS Clinical Trials Group criteria. [13] Several studies have suggested an interest in thalidomide for treating AIDS-related KS because of its anti-angiogenic and immunomodulatory properties. [10]

KS can be managed with a conservative approach and discontinuation of the immunosuppressive treatment. However, discontinuation of the immunosuppression is not always possible and, in such cases where immunosuppression is discontinued, chemotherapy may be indicated. [14] In our patient, withdrawal of immunosuppression improved Kaposi skin lesions but induced a relapse of alveolar hemorrhage that is rare in ANCA vasculitis patients undergoing chronic HD.

   References Top

1.Catricalà C, Marenda S, Muscardin LM, Donati P, Lepri A, Eibenschutz L. Angiomatous reaction Kaposi-sarcoma-like as a side effect of topical corticosteroid therapy in lichen sclerosus of the penis. Dermatol Ther 2009;22:379-82.  Back to cited text no. 1
2.Tedesco M, Benevolo M, Frezza F, et al. Colorectal Kaposi's sarcoma in an HIV-negative male in association with ulcerative rectocolitis: A case report. Anticancer Res 1999;19:3045-8.  Back to cited text no. 2
3.de Sanjose S, Mbisa G, Perez-Alvarez S, et al. Geographic variation in the prevalence of Kaposi sarcoma-associated herpesvirus and risk factors for transmission. J Infect Dis 2009;199:1449-56.  Back to cited text no. 3
4.Pastor MA, Vasco B, Mosquera JM, Debén G, Bautista P, Requena L. Two HHV8-related illnesses in a HIV-negative patient: Kaposi's sarcoma and multicentric Castleman's disease. Response to treatment with Rituximab and CHOP. Actas Dermosifiliogr 2006;97:385-90.  Back to cited text no. 4
5.Aseni P, Vertemati M, Minola E, et al. Kaposi's sarcoma in liver transplant recipients: Morphological and clinical description. Liver Transpl 2001;7:816-23.  Back to cited text no. 5
6.Ahmadpoor P. Human herpesvirus-8 and kaposi sarcoma after kidney transplantation: Mechanisms of tumor genesis. Iran J Kidney Dis 2009;3:121-6.  Back to cited text no. 6
7.Magri MC, Carbone PH, De Los Santos-Fortuna E, Caterino-de-Araujo A. A comparative study of the frequency of antibody and titers against human herpesvirus 8 latent and lytic antigens in "at-risk" individuals and among patients with Kaposi's sarcoma. J Med Virol 2009;81:1292-7.  Back to cited text no. 7
8.Monti M, Mancini LL, Ceriani R, Hendrickx I, Guizzardi M. Iatrogenic Kaposi's sarcoma and HCV infection. J Eur Acad Dermatol Venereol 2004;18:191-3.  Back to cited text no. 8
9.Tiruneh M. Seroprevalence of multiple sexually transmitted infections among antenatal clinic attendees in Gondar Health Center, northwest Ethiopia. Ethiop Med J 2008;46:359-66.  Back to cited text no. 9
10.Ben M'barek L, Fardet L, Mebazaa A, et al. A retrospective analysis of thalidomide therapy in non-HIV-related Kaposi's sarcoma. Dermatology 2007;215:202-5.  Back to cited text no. 10
11.Bacha MM, Goucha R, Zouaghi K, et al. Myeloma, Kaposi's sarcoma and HHV8 infection in hemodialyzed patient. Tunis Med 2007;85: 237-9.  Back to cited text no. 11
12.Hauerstock D, Gerstein W, Vuong T. Results of radiation therapy for treatment of classic Kaposi sarcoma. J Cutan Med Surg 2009;13:18-21.  Back to cited text no. 12
13.Silvestris N, Galetta D, Colucci G. Successful treatment with three-weekly paclitaxel of an anthracycline-refractory classical Kaposi's sarcoma. Anticancer Res 2009;29:675-6.  Back to cited text no. 13
14.Svrcek M, Tiret E, Bennis M, Guyot P, Fléjou JF. KSHV/HHV8-associated intestinal Kaposi's sarcoma in patient with ulcerative colitis receiving immunosuppressive drugs: Report of a case. Dis Colon Rectum 2009;52:154-8.  Back to cited text no. 14

Correspondence Address:
Lilia Ben Fatma
Department of Nephrology, La Rabta Hospital, Jabbari, 1007, Tunis
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.121285

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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