Autori: A. Cappoli1,2, F. Maellaro1,2, K. Korreshi1,2, I. Umbro1, G. Gentile4, F. Tinti1, S. Lai1.2, S. Mazzaferro2,3, A.P. Mitterhofer1,2
Affiliazioni: Dipartimento di Medicina Clinica1, Scuola di Specializzazione in Nefrologia e UOC di Nefrologia2, Dipartimento di Scienze Cardiovascolari, Respiratorie, Nefrologiche, Anestesiologiche e Geriatriche3, Dipartimento di Biotecnologie Cellulari e Ematologia4, Policlinico Umberto I, Sapienza Università di Roma.
Background: Castleman’s disease (CD) is a rare lymphoproliferative disorder with two clinical variants. Human Herpes Virus 8 (HHV-8) has been associated with multicentric CD (MCD). MCD was primarily described in human immunodeficiency virus (HIV)–infected patients but, recently, HHV-8 has been detected in HIV-negative patients.
Case report: A 62 yrs-old man received a deceased donor renal transplant (tx) in 2004 and started immunosuppressive therapy. Six years after tx he developed renal dysfunction with fever, night sweats, weakness, fatigue and anorexia. Laboratory findings on admission showed renal failure (creatinine 2.6 mg/dL), anaemia (8.3 g/dL), monoclonal gammopathy, normal white blood cell count and increased C-Reactive Protein. A full body CT scan revealed enlarged lymph nodes, hepatomegaly and splenomegaly. The patient underwent a bone marrow biopsy which was normal, and to the histological examination of a laterocervical lymph node that revealed follicles with transformed germinal centres with strong immunoreactivity for HHV-8, diagnostic for plasmablastic MCD. High HHV-8 DNA viral load was detected (480.000 copies/mL) with HIV infection negative. Immunosuppressive therapy was decreased and ganciclovir was started. The patient was treated with anti-CD20 antibodies with a quick reduction of HHV-8 viral load but died shortly after.
Conclusion: Multicentric Castleman’s Disease is a complication of immunosuppressive therapy in kidney transplanted patients, difficult to diagnose and frequently unrecognized since its signs and symptoms are unspecific. Nephrologists should be alert for this disease in transplant recipients because of the high rate of mortality and therapy should be initiated promptly.