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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2019  |  Volume : 30  |  Issue : 2  |  Page : 537-539
Kaposi sarcoma development following microscopic polyangiitis treatment

1 Department of Internal Medicine, University of Health Sciences Antalya Training and Research Hospital, Antalya, Turkey
2 Department of Nephrology, University of Health Sciences Antalya Training and Research Hospital, Antalya, Turkey

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Date of Submission31-Mar-2018
Date of Decision15-May-2018
Date of Acceptance07-Feb-2018
Date of Web Publication23-Apr-2019


Kaposi sarcoma (KS) is a rare condition mostly seen in immunosuppressed patients due to a syndrome or organ transplantation. However, few cases have been reported in patients with rheumatologic diseases treated with long-term corticosteroid and cyclophosphamide. We present a case in which the subject developed KS following a course of immunosuppressive therapy for their systemic vasculitides.

How to cite this article:
Biricik M, Eren M, Bostan F, Yılmaz &, İnci A. Kaposi sarcoma development following microscopic polyangiitis treatment. Saudi J Kidney Dis Transpl 2019;30:537-9

How to cite this URL:
Biricik M, Eren M, Bostan F, Yılmaz &, İnci A. Kaposi sarcoma development following microscopic polyangiitis treatment. Saudi J Kidney Dis Transpl [serial online] 2019 [cited 2022 Nov 26];30:537-9. Available from: https://www.sjkdt.org/text.asp?2019/30/2/537/256864

   Case Report Top

A 71-year-old male patient with hypertension was admitted to the emergency department with bloody sputum and shortness of breath. The patient’s vitals were BP: 140/70 mm Hg, pulse rate: 78/min, respiratory rate: 15/min, and body temperature: 36.7°C. Respiratory rates in both lungs reaching middle zones and pretibial edema were noted in physical examination. His laboratory test results were as follows: C-reactive protein: 61 mg/L, erythrocyte sedimentation rate: 83 mm/h, blood urea nitrogen: 39 mg/dL, creatinine: 4.1 mg/dL, hemoglobin: 10.1 g/dL, serum albumin: 3.1 g/dL, intact parathyroid hormone: 96 pg/mL, ferritin: 207 ng/mL, urinalysis: WBC: 10 P/HPF, RBC: 95 P/HPF, protein/creatinine ratio in spot urine: 5.15 g/dL, ANA (-), and p-ANCA (+++). In venous blood analysis, pH was 7.41, HCO3 was 22.6 mEq/L, and pCO2 was 40 mm Hg. HbsAg, anti-HIV 1–2, and anti-HCV tests were nonreactive. Both kidneys were in normal sizes in urinary ultrasound imaging, and Grade 2 increase in renal parenchymal echogenicity was observed. Ground-glass appearance in patchy pattern and thin reticular opacities were present in chest X-ray and high-resolution computed tomography of the chest [Figure 1]. Pulmonary-renal syndrome was considered in this patient, so a renal biopsy was performed. The biopsy exhibited pauci-immune necrotizing glomerulonephritis. Diagnosed with mPAN, regimens of pulse steroid (1 g/day, 3 days), plasmapheresis (eight times, every other day), and three cycles of intravenous cyclophosphamide (500 mg/ m2/month) was planned and administered to patient. Respiratory system findings regressed during follow-ups; however, renal function tests remained stable and hemodialysis scheduled for the patient three times a week. At the 1st month control, after the third cyclophosphamide treatment, purple raised skin lesions on plantar sites of both feet and left leg were noticed on physical examination [Figure 2] and [Figure 3]. Biopsies obtained from these skin lesions revealed Kaposi sarcoma (KS). As the patient evaluated for the etiology of KS, his serology tests and skin biopsy material were both positive for human herpes virus type-8 (HHV-8). Hence, the cyclophosphamide treatment for vasculitis was discontinued, and the steroid dose given was decreased. The patient was consulted to medical oncology and radiation oncology departments. Radiation therapy to sites of lesions was offered to the patient by related departments. The patient was transferred to radiation oncology clinic for radiation therapy. The lesions regressed after decreasing the immunosuppressive therapy.
Figure 1: Ground-glass appearance in patchy pattern and thin reticular opacities.

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Figure 2: Purple raised skin lesion on the left leg.

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Figure 3: Purple raised skin lesion on the plantar site of the left foot.

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Informed consent was obtained from the patient before reporting the case.

   Discussion Top

KS presents with brownish, red-to-bluish cutaneous nodules that tend to enlarge into dome-shaped tumors, especially at distal parts of lower extremities, heels, and feet mostly.[1],[2],[3] KS development following immunosuppressive therapy in rheumatologic patients is a rarely reported condition. In literature, four cases of granulomatous polyangiitis and one case of mPAN were previously reported to develop KS.[4],[5],[6],[7],[8] In the case of KS development following mPAN treatment, the patient was admitted with pulmonary renal syndrome, and the lesions appeared lower extremities, head and neck at the 5th month of cyclophosphamide therapy. The patient was diagnosed with KS following skin biopsies. HHV-8 was positive both in blood samples and skin lesions. The lesions regressed after decreasing immunosuppressive doses rapidly.[9] KS is seen commonly in regions endemic for HHV-8. HHV-8 virus reactivates with immunosuppression and shows its oncogenic effects by suppressing p53 and retinoblastoma tumor suppressor proteins. Unless immunosuppressive therapy is given up, KS progression continues.[7]

First step of treating iatrogenic KS is discontinuation or decreasing doses of immunosuppressive therapy. In two cases of KS developing after granulomatous polyangiitis treatment (steroid + cyclophosphamide), the lesions regressed following discontinuation of immunosuppressive therapy.[4],[5] In another case, the lesions did not regress after discontinuation of immunosuppressive therapy. Moreover, increase in the number of the lesions, pain, edema, and paresthesia observed so intravenous doxorubicin therapy was planned.[7] It is reported that doxorubicin shows its effects by decreasing HHV-8 viremia and also by altering cytokine production in infected cells.[9] In our case, the lesions regressed following decreasing immunosuppressive doses, and radiation therapy was performed. Skin lesions of KS are radio sensitive, and radiotherapy increases patient’s quality of life with minimum toxicity.

There are some evidence suggesting steroids stimulate tumor development and growth. Gou et al reported KS lesions include steroid receptors in high amounts, and these receptors can be stimulated with exogenous steroids and inflammatory cytokines.[10]

   Conclusion Top

In patients receiving immunosuppressive agents, careful skin examination is required as they are prone to develop skin cancers including KS. This case aims to contribute clinical literature about treating this kind of diseases.

Conflicts of interest: None declared.

   References Top

Antman K, Chang Y. Kaposi’s sarcoma. N Engl J Med 2000;342:1027-38.  Back to cited text no. 1
Schwartz RA. Kaposi’s sarcoma: An update. J Surg Oncol 2004;87:146-51.  Back to cited text no. 2
Louthrenoo W, Kasitanon N, Mahanuphab P, Bhoopat L, Thongprasert S. Kaposi’s sarcoma in rheumatic diseases. Semin Arthritis Rheum 2003;32:326-33.  Back to cited text no. 3
Deschenes I, Dion L, Beauchesne C, de Brum-Fernandes A. Kaposi’s sarcoma following immunesuppressive therapy for Wegener’s granulomatosis. J Rheumatol 2003;30:622-64.  Back to cited text no. 4
Erban SB, Sokas RK. Kaposi’s sarcoma in an elderly man with Wegener’s granulomatosis treated with cyclophosphamide and corticosteroids. Arch Intern Med 1988;148:1201-3.  Back to cited text no. 5
Hoff M, Rødevand E. Development of multiple malignancies after immunosuppression in a patient with Wegener’s granulomatosis. Rheumatol Int 2005;25: 238-40.  Back to cited text no. 6
Saxena A, Netchiporouk E, Al-Rajaibi R, Billick R, Roshdy O. Iatrogenic Kaposi’s sarcoma after immunosuppressive treatment for granulomatosis with polyangiitis (Wegener’s). JAAD Case Rep 2015;1:71-3.  Back to cited text no. 7
Fatma LB, Rais L, Mebazza A, et al. Kaposi’s sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient. Saudi J Kidney Dis Transpl 2013;24:1199-202.  Back to cited text no. 8
[PUBMED]  [Full text]  
Uneda S, Murata S, Sonoki T, Matsuoka H, Nakakuma H. Successful treatment with liposomal doxorubicin for widespread Kaposi’s sarcoma and human herpesvirus-8 related severe hemophagocytic syndrome in a patient with acquired immunodeficiency syndrome. Int J Hematol 2009; 89:195-200.  Back to cited text no. 9
Guo WX, Antakly T. AIDS-related Kaposi’s sarcoma: evidence for direct stimulatory effect of glucocorticoid on cell proliferation. Am J Pathol 1995;146:727-34.  Back to cited text no. 10

Correspondence Address:
Merve Eren
Department of Internal Medicine, Antalya Training and Research Hospital, Antalya
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.256864

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  [Figure 1], [Figure 2], [Figure 3]

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