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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2011  |  Volume : 22  |  Issue : 2  |  Page : 319-323
Kaposi's sarcoma following immunosuppressive therapy for vasculitis

1 Unit of Nephrology, Dialysis, Renal Transplantation, Ibn Sina University Hospital, Rabat, Morocco
2 Laboratory of Pathologic Anatomy, HER - CHU Ibn Sina, Rabat, Morocco
3 Unit of Dermatology, Ibn Sina University Hospital, Rabat, Morocco

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Date of Web Publication18-Mar-2011


Kaposi's sarcoma (KS) is widely reported to develop after renal transplantation and is induced by activation of a latent human herpes virus 8. We report the clinical features and outcome of a 50-year-old woman who presented with KS 18 weeks after starting immuno­suppressive therapy for vasculitis. She had positive-titer IgG antibody to human herpes virus 8. Cyclophosphamide pulses were interrupted, and prednisone was decreased gradually to 10 mg/day. Skin lesions showed important regression with stabilization of the general state and renal function. Eight months later, the patient presented with a diffuse cutaneous KS that required the discontinuation of steroids. Within 1 month, her general status and renal function deteriorated, and she died with a disseminated intravascular coagulation syndrome.

How to cite this article:
Bouattar T, Kazmouhi L, Alhamany Z, Beqqal K, Haffane L, Houssaini TS, Rhou H, Benamar L, Senouci K, Bayahia R, Ouzeddoun N. Kaposi's sarcoma following immunosuppressive therapy for vasculitis. Saudi J Kidney Dis Transpl 2011;22:319-23

How to cite this URL:
Bouattar T, Kazmouhi L, Alhamany Z, Beqqal K, Haffane L, Houssaini TS, Rhou H, Benamar L, Senouci K, Bayahia R, Ouzeddoun N. Kaposi's sarcoma following immunosuppressive therapy for vasculitis. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2021 Oct 22];22:319-23. Available from: https://www.sjkdt.org/text.asp?2011/22/2/319/77618

   Introduction Top

Kaposi's sarcoma (KS), a vascular malignant tumor, is an endemic disease in some areas of Africa and is frequently associated with ac­quired immunodeficiency syndrome. [1],[2] It also develops in patients who receive corticoste­roids (CS) or immunosuppressive therapy for many illnesses or for organ transplantation. [3],[4],[5],[6],[7],[8],[9]

We report a case of iatrogenic KS in a patient with vasculitis treated with immunosuppressive therapy.

   Case Report Top

A-51-year-old woman of Mediterranean ori­gin, without pathological antecedents, was ad­mitted in our department for alteration of the general status with severe dyspnea. On admi­ssion, the clinical examination disclosed a con­scious patient with fever (38.3°C), oligoanuria, and active urinary sediment (albumin: ++, ery­throcyte: ++). Her blood pressure was 80/40 mm Hg, heart rate was 110 beats/min, and res­piratory rate was 35 beats/min. The initial in­vestigations revealed the following: blood urea 78.85 mmol/L, serum creatinine 2217 μmol/L, bicarbonate level 5 meq/L, hemoglobin 6.9 g/ dL, and lactate dehydrogenase (LDH) 930 IU/L.

C-reactive protein was 159 mg/L and urine culture was positive for  Escherichia More Details coli.

The patient received several sessions of he­modialysis using a right jugular catheter. The septic shock was managed by treatment with ceftriaxone 2 g/d for 21 days. Renal ultrasono­graphy showed normal sized kidneys. Later, the renal biopsy showed a pauci-immune nec­rotizing and crescentic glomerulonephritis. Fur­ther serologic investigations revealed decreased complement C3 (0.78 IU/L) and positive anti­nuclear antibodies, anti-phospholipid antibodies, and antineutrophil cytoplasmic antibodies (C­ANCA).

The Ear-Nose-Throat exam showed ulcera­tions of the left nose, corresponding to nonspe­cific subacute inflammatory change in biopsy.

The patient received a pulse of methyl pred­nisolone followed by oral prednisone 60 mg/ day for 6 weeks that was gradually tapered off in addition to monthly pulses of cyclophos­phamide. During the follow-up, the renal func­tion improved with serum creatinine 410 μmol/ L, serologic tests normalized, and anemia was corrected.

Eighteen weeks after receiving a total dose of 5,125 g of CS and 2,975 g of cyclophospha­mide and 10 days after the fifth cyclophospha­mide pulse, the patient developed cutaneous infiltrating maculopapular and purplish lesions in the upper part of the two forearms, the ante­rior face of the chest, and the back and of both legs [Figure 1]. The skin biopsy was compa­tible with a KS [Figure 2], [Figure 3], and [Figure 4]. The blood investigation revealed a positive titer of the IgG antibodies of human herpes virus 8 (HHV8) and negative serology of HIV. The chest X-ray was unremarkable. Accordingly, the cyclophos­phamide pulse was interrupted and prednisone decreased to 10 mg/day.
Figure 1: Cutaneous infiltrating maculopapular and purplish lesions in the upper part of the left leg.

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Figure 2: Purplish nodules in the right forearm.

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Figure 3: Cutaneous nodule, coloration HE: fusiform proliferation richly vascularized.

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Figure 4: Immunohistochemical labeling with CD 31, positivity on the vascular walls and isolated cells.

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After seven months, there was evidence of important regression of the cutaneous lesions with stabilization of the general status and re­nal function.

Eight months later, the patient presented with a cutaneous diffuse form of KS that required the progressive tapering of the CS. In one month, the evolution was marked by a deterioration of the general state and renal function which ne­cessitated the restart of the hemodialysis. Even­tually, the patient died with a disseminated intravascular coagulation syndrome.

   Discussion Top

Our patient developed KS 18 weeks after receiving a high cumulative dose of steroids and cyclophosphamide. Louthrenoo et al re­viewed 24 cases of KS associated with treat­ment of rheumatic disease, [10] and since 2003, a few other cases have been reported, including a Moroccan woman who developed KS after cyclophosphamide and CS treatment for lupus nephritis. [11]

Up till now, a few cases of KS in patients with glomerulonephritis have been described. In one case, a membranous glomerulonephritis was found and a month later KS appeared when CS or immunosuppressive therapy was discon­tinued. [12] In a case of membranoproliferative glomerulonephritis and mild skin KS at the same time started in a 46-year-old white male of Italian origin; KS rapidly developed in the following months after the patient received prednisolone. [13] A case of KS was reported in a patient with focal glomerulosclerosis after treat­ment with cyclosporine, steroids and cyclo­phosphamide. [14] Furthermore, there are three published case reports of KS development after immunosuppressive therapy for Wegener's granulomatosis. [15],[16],[17]

Our patient described here developed KS 18 weeks after the beginning of immunosuppres­sive therapy. The reason for this early presen­tation might be the ethnic background of this patient. The Maghreb region, especially Moro­cco, is considered to be an endemic area for classical KS. [18]

The true nature of KS, either hyperplasia or neoplasia, remains controversial. In 1994, Chang et al [19] described a novel herpes virus from a KS lesion in an HIV-positive patient which was named HHV 8. It was subsequently isolated from all types of KS lesions, including CS-induced KS. [20] A preliminary study showed that HHV8 infection is endemic in the general population of Morocco and that serological tests were positive for latent HHV8 antibodies in most KS cases. [21] Our patient had a positive HHV 8 serology. HHV8 seems to be necessary but not sufficient to induce KS, since it was noticed in transplant patients who were HHV8 positive before transplantation, but the tumor occurred only with posttransplant immune sup­pression.

In previously reported cases, CS was the main factor for the development of KS and cyclophosphamide made it worse. How can immunosuppressive treatment lead to the emer­gence of KS? In vitro some evidence supports the hypothesis that steroids have a direct role in stimulating tumor development and growth. [22] Guo et al [23] studied KS cells from individuals with HIV and showed that steroids stimulate their proliferation. They showed that steroid receptors are present at high levels on KS le­sions and that receptors can be up-regulated by exogenous steroids and inflammatory cytokines. Hudnall et al [24] have reported increased viral replication and activation of the lytic cycle of HHV8 after steroid administration in trans­fected cells. Therefore, the effects of steroids are twofold: the up-regulation of KS cells pro­liferation and the activation of HHV8. None­theless, the majority of patients placed on CS or immunosuppressive therapy do not develop KS. This observation supports the hypothesis that the development of KS may require fur­ther ethnic, geographical, genetic or environ­ mental predisposing factors. [25],[26]

Spontaneous remission of KS is usual after discontinuation of CS. Moreover, in some ca­ses, KS lesions resolved once prednisone the­rapy was decreased or stopped despite conti­nuing administration of low dose of cyclophos­phamide. [16] The occurrence of KS in a patient with glomerulopathy raises a therapeutical di­lemma: interrupting immunosuppressive the­rapy improves the KS lesions but can be fol­lowed by a deterioration of renal function. In our case, the evolution was unfavorable and the patient died by a disseminated intravascular coagulation syndrome one month after the ste­roids were stopped.

   References Top

1.Centers for Disease Control. Kaposi's sarcoma and pneumocystis pneumonia among homo­sexual men-New York City and California. MMWR Morb Mortal Wkly Rep 1981;25:305­-8.  Back to cited text no. 1
2.Centers for Disease Control. Epidemiologic aspects of the current outbreak of Kaposi's sarcoma and opportunistic infections. N Engl J Med 1982;306:248-52.  Back to cited text no. 2
3.Penn I. The changing pattern of post transplant malignancies. Transplant Proc 1991;23:1101-3.  Back to cited text no. 3
4.Martin RW III, Hood AF, Farmer ER. Kaposi's sarcoma. Medicine 1993;72:245-61.  Back to cited text no. 4
5.Sanders C, Canninga-van Dijk M. Kaposi's sarcoma. Lancet 2004,364:1549- 52.  Back to cited text no. 5
6.Antman K, Chang Y. Kaposi's sarcoma. N Engl J Med 2000;342:1027-38.  Back to cited text no. 6
7.Hengge UR, Ruzicka T, Tyring SK, et al. Update on Kaposi's sarcoma and other HHV8 associated diseases. Part 1: epidemiology, en­vironmental predispositions, clinical manifes­tations, and therapy. Lancet Infect Dis 2002; 2:281-92.  Back to cited text no. 7
8.Rappersberger K, Stingl G, Wolff K. Kaposi's sarcoma. In: Freedberg IM, Sisen AZ, Wolff K, et al, eds. Fitzpatrick's Dermatology in Ge­neral Medicine, 5th ed. New York, McGraw­Hill 1999;1195-204.  Back to cited text no. 8
9.Farge D. Kaposi's sarcoma in organ transplant recipients. The Collaborative Transplantation Research Group of Ile de France. Eur J Med 1993;2:339-43.  Back to cited text no. 9
10.Louthrenoo W, Kasitanon N, Mahanuphab P, Bhoopat L, Thongprasert S. Kaposi's sarcoma in rheumatic disease. Semin Arthritis Rheum 2003;32:326-33.  Back to cited text no. 10
11.El Maghraoui A, Sekkach Y, Quacif H, et al. Iatrogenic Kaposi's sarcoma following immu­nosuppressive therapy for systemic lupus ery­thematosus. Clin Exp Rheumatol 2003;21:674.  Back to cited text no. 11
12.Barics YS, Akpolat T, Akpolat I, Karagoz F, Kandemir B. Coexistence of membranous glo­merulonephritis and Kaposi's sarcoma (letter). Nephron 1998;79:371-2.  Back to cited text no. 12
13.Freuler CB, Durlach RA, Toblli JE, Cicco J, Casas J. Kaposi's sarcoma in young adults without evidence of HIV infection. Medicina (B-Aires) 1994;54:145-9.  Back to cited text no. 13
14.Zerbi S, Saruggia M, Brambilla L, Lodeville D, Buccianti G. Kaposi's sarcoma in a patient with focal glomerulosclerosis. J Nephrol 2001; 14:299-303.  Back to cited text no. 14
15.Deschenes I, Dion L, Beauchesne C, Brum­Fernandes A. Kaposi's sarcoma following immune suppressive therapy for Wegener's granulomatosis. J Rheumatol 2003;30(3):622-­4.  Back to cited text no. 15
16.Erban S, Sokas R. Kaposi's sarcoma in an elderly man with Wegener's granulomatosis treated with cyclophosphamide and cortico­steroids. Arch Intern Med 1988;148(5):1201-3.  Back to cited text no. 16
17.Hoff M, Rodevand E. Development of multiple malignancies after immunosuppression in a patient with Wegener's granulomatosis. Rheumatol Int 2005;25(3):238-40.  Back to cited text no. 17
18.Hbid O, Belloul L, Fajali N, et al. Kaposi's sarcoma in Morocco: a pathological study with immunostaining for human herpesvirus-8 LNA­1. Pathology 2005;37:288-95.  Back to cited text no. 18
19.Chang Y, Cesarman E, Pessin E. Identification of herpesvirus-like sequences in AIDS asso­ciated kaposi's sarcoma. Science 1994;266: 1865-9.  Back to cited text no. 19
20.Rady PL, Hodak E, Yen A. Detection of human herpesvirus-8 DNA in Kaposi's sarcomas from iatrogenically immunosuppressed patients. J Am Acad Dermtol 1998;38:429-37.  Back to cited text no. 20
21.El Kassimi B, Benchemsi N, Mikou O, El Ouazzani T, Lakhdar H. Kaposi's sarcoma and anti-herpes virus 8 antibodies in Morocco. Médecine et maladies infectieuses 2003;33: 226-8.  Back to cited text no. 21
22.Cai J, Zheng T, Lotz M, Zhang Y, Masood R, Gill P. Glucocorticoids induce Kaposi's sar­coma cell proliferation through the regulation of transforming growth factor-b. Blood 1997; 89(5):1491-500.  Back to cited text no. 22
23.Guo W, Antakly T. AIDS-related Kaposi's sarcoma: evidence for direct stimulatory effect of glucocorticoid on cell proliferation. Am J Path 1995;146:727-34.  Back to cited text no. 23
24.Hudnall S, Rady P, Tyring S, Fish J. Hydro­cortisone activation of human herpesvirus-8 viral DNA replication and gene expression in vitro. Transplantation 1999;67:648-52.  Back to cited text no. 24
25.Selvi E, Stefano R, Manganelli S. Kaposi's sarcoma in psoriatic arthritis. Rheumatology 2003;42:389.  Back to cited text no. 25
26.Mezalek ZT, Harmouche H, Attar NE, et al. Kaposi's sarcoma in association with Behcet's Disease: Case report and literature Review. Semin Arthritis Rheum 2006;36:328-31.  Back to cited text no. 26

Correspondence Address:
Tarik Bouattar
Unit of Nephrology, Dialysis, Renal Transplantation, Ibn Sina University Hospital, Rabat
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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