Kaposi's sarcoma occurring in a young black man after kidney transplantation
Antonietta De Blasio,
Giuseppe Palmiero and
Domenico Russo
Department of Nephrology, School of Medicine, University Federico II, Naples, Italy
Correspondence and offprint requests to: Domenico Russo, MD, Via Marconi, 80, 80024 Cardito. Napoli, Italy. Email: domenicorusso51{at}hotmail.com
Keywords: Kaposi's sarcoma; kidney transplantation; gastro-intestinal bleeding
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Introduction
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Kaposi's sarcoma (KS) is a cancer of connective and fibrous tissue such as cartilage, bone, fat, muscle, blood vessels, tendons and ligaments [13]. It was first described by dermatologist Moritz Kaposi as a disease characterized by ... idiopathic multiple pigmented lesions of the skin ... . For decades KS has been regarded as an uncommon disease that mostly affects elderly men of Mediterranean or Jewish heritage, young adult African men and organ transplanted patients [1].
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Case report
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Here we describe a case of a 42-year-old black African male who developed KS a few months after renal transplantation.
The cause and the onset of renal insufficiency was unknown. Haemodialysis treatment started in 1992. Clinical history during renal replacement therapy was unremarkable. In January 2002, the patient underwent a cadaveric renal transplantation; the donor was a black man coming from Africa (Nigeria). The other kidney was shipped to another Transplantation Unit and was not implanted for unknown reason. The pre-transplant serology showed negative tests for hepatitis B surface antigen and for anti-HCV as well as HIV. High plasma levels of specific immunoglobulin G were found indicating previous infections by cytomegalovirus, herpes simplex, varicella-zoster and Epstein-Barr. Human Herpes virus 8 was not detected in the donor and the recipient. No complications were encountered during the transplantation procedures. Polyclonal antilymphocyte sera were not administered. Diuresis restarted after 20 days. At the time of discharge, serum creatinine was 185.64 µmol/l, GFR 0.87 ml/s; immunosuppressive therapy consisted of: prednisone, 16 mg/day; tacrolimus (FK 506), 24 mg/day; mycophenolate (MMF), 2 g/day. In May 2002, five months after transplantation, the patient was admitted to our department because of abdominal pain. Clinical examination and laboratory work-up were unremarkable. No fever was present. The upper gastrointestinal endoscopy showed signs of gastritis and duodenitis. Ultrasonography did not reveal alteration of abdominal organs. There was no evidence of skin lesions. The patient was discharged and followed-up. A few weeks after the discharge, the patient was re-admitted to the hospital because of recurrent severe abdominal pain and rapid weight loss due to nausea (4 kg in one week). Routine biochemistry was negative but serum creatinine was 362.44 µmol/l. Pitting oedema of legs was present. At physical examination, an inguinal lymph node was found; biopsy showed spindle cells, a vascular space intermingled with some networks of blood filled slits, flattened endothelial cells and macrophages (Figure 1A and B). Immunohistochemical reaction with CD34 was positive. The patient underwent total body CT scan which showed multiple round lesions on the liver and an enormous increase in the thickness of the body of the stomach (Figure 2). The upper gastrointestinal endoscopy was repeated and two lesions were found on pyloric antrum (Figure 3) and one more on the first portion of the duodenum; histologic exam revealed alterations similar to those found in the inguinal lymph node. The immunosuppressive therapy was firstly tapered and thereafter withdrawn; contemporaneous Doxorubicina therapy (35 mg per venam every two weeks) was started. Renal function rapidly worsened requiring some haemodialysis sessions. Three weeks after admission, anaemia (Hb 37 g/l) ensued because of severe gastrointestinal bleeding; the consequent paleness of skin made multiple lesions on trunk, legs and arms visible. Several blood transfusions were unable to correct the anaemia and restore a stable haemodynamic condition; thus a sub-total gastrectomy was performed. Biopsy revealed histological alterations similar to those found in the inguinal lymph node. The patient died few days after surgery.

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Fig. 1 (A and B). Spindle cells, vascular space intermingled with some networks of blood filled slits, flattened endothelial cells and macrophages.
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Fig. 2. Abdominal CT scan shows: (A) multiple round lesions on liver; (B) enlarged gastric wall and pancreas calcification.
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Discussion
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Different subtypes of KS have been described [4,5]:
- Classic KS that develops in Jewish men of Eastern European countries or among men of Mediterranean heritage (mainly Italian) between the ages of 50 and 70.
- African, endemic, KS, that develops in young people living in equatorial Africa.
- AIDS-related, epidemic, KS that arises in people who are infected with HIV. Nowadays, this is the most frequent cause of KS due to the spreading of acquired immunodeficiency syndrome (AIDS).
- Transplant-related (acquired) KS.
Transplant-related KS is increasingly encountered as a consequence of the long-lasting suppression of the immune system [6,7]. The institution of cyclosporine as extensive therapy in transplantation is regarded as the most important pathogenetic factor that may account for the increased prevalence of KS in transplanted patients [1,810]. Therapy with polyclonal antilymphocyte sera may also be responsible for the appearance of KS [8,9]. Besides immunosuppressive therapy, genetic factors may play a pathogenetic role since prevalence of KS is higher in Mediterranean, Caribbean and black African patients; for instance, the risk of KS is 224-fold higher in Italian transplanted patients [1]. Finally, KS may be caused by human herpes virus 8 [3,6,10,11].
The case of KS described herein presents some peculiarities such as the early onset and the extremely fast progression even in absence of cyclosporine and/or polyclonal antilymphocyte sera therapy, the contemporaneous and prompt cutaneous, lymphatic and visceral involvement, the major gastrointestinal bleeding ending with exitus. The average interval between transplantation and the onset of KS is 20 months with the shortest interval in cyclosporine-treated recipients [1]. The long-lasting suppression of the immune system mainly with cyclosporine is likely the most important pathogenetic factor [1,810]; in fact, complete regression of the disease is obtained by tapering or replacing cyclosporine therapy. In our patient, KS appeared five months after renal transplantation and in the absence of cyclosporine therapy. The scheduled immunosuppressive therapy was prednisone, FK 506, and MMF; the latter medication is usually prescribed to replace cyclosporine in patients who develop KS [8]. Nonetheless, a case of KS appearing after a seven-year treatment with MMF has been reported [8]; however, that patient had been treated with cyclosporine for a long time before, and therapy with MMF was instituted when cutaneous KS had already appeared. It was more likely a case of a late recurrence rather than a de novo onset of KS.
Skin lesions are encountered in more than 90% of KS; less frequent is the involvement of viscera. Cutaneous lesions have a dark blue or purplish color on white skin and appear as lightly pigmented areas on black skin. In the latter case, they may be missed at clinical examination. Leg oedema often pre-dates skin lesions by months. In our patient, peripheral pitting, oedema was evidenced at 2nd admission to hospital, while skin lesions became evident only during the days of severe anaemia. Therefore, the risk of missing cutaneous lesion of KS on black skin should be taken into the account.
The most impressive feature of the present case is the fast and simultaneous cutaneous, lymphatic and visceral involvement. Visceral lesions due to KS are mostly observed in stomach and duodenum. In the present case, a few weeks after an initial negative assessment, lesions were found on liver, stomach and duodenum; thus, the patient shortly reached stage 3, in the four-stage classification of KS [1,12]. The gastrointestinal lesions are generally asymptomatic and rarely responsible for major bleeding or perforation [2]. In contrast, in our patient, long-lasting bleeding with consequent severe anaemia was observed.
Racial factors may explain the fast and fatal progression seen in our case. It is likely that the patient (or the donor?) had African (endemic) KS, per se more aggressive and with worse prognosis, rather than a transplant-related (acquired) KS; the suppression of the immune system for a few months (even without cyclosporine therapy) may have prompted the appearance and worsened the course of the disease.
In conclusion, KS may develop in renal transplant patient even when not treated with cyclosporine and may have a fast and fatal progression in presence of racial factors.
Conflict of interest statement. None declared.
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References
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Received for publication: 29. 7.05
Accepted in revised form: 6. 9.05