Autopsy and Case Reports
Autopsy and Case Reports
Autopsy Case Report

Two cases of rare HHV8-driven intravascular lymphoma with synchronous Kaposi sarcoma, both diagnosed at autopsy in renal transplant recipients.

Paida Gwiti; Megan Jenkins; Judit Sutak; Zsombor Melegh

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ABSTRACT: We present the first report of two rare yet remarkably similar autopsy cases of Kaposi sarcoma (KS) and intravascular human herpesvirus 8 (HHV8) positive lymphoproliferative disorder in renal transplant patients. It is well established that HHV8 infection causes Kaposi sarcoma (KS). More recently, it is recognized that HHV8 is also related to several lymphoproliferative conditions. These are poorly characterized and often difficult to diagnose. In both cases described herein, the diagnoses of multifocal hepatic KS and intravascular HHV8 positive (EBV negative) systemic diffuse large B-cell lymphoma, NOS were made at autopsy. Given the findings we describe in cases with fatal outcomes, we discuss the implications of HHV8 screening in solid allograft recipients.


Herpesvirus 8, Human, Sarcoma, Kaposi, Autopsy, Transplantation, Lymphoproliferative disorder, Intravascular lymphoma


1 Chang Y, Cesarman E, Pessin MS, et al. Identification of herpesvirus-like DNA sequences in AIDS-associated Kaposi’s sarcoma. Science. 1994;266(5192):1865-9. PMid:7997879.

2 Naresh KN, Rice AJ, Bower M. Lymph nodes involved by multicentric Castleman disease among HIV-positive individuals are often involved by Kaposi sarcoma. Am J Surg Pathol. 2008;32(7):1006-12. PMid:18469709.

3 McAllister SC, Moses AV. Endothelial cell- and lymphocyte-based in vitro systems for understanding KSHV biology. Curr Top Microbiol Immunol. 2007;312:211-44. PMid:17089799.

4 Schulz TF. Kaposi's sarcoma-associated herpesvirus (human herpesvirus 8): Epidemiology and pathogenesis. J Antimicrob Chemother. 2000;45(Suppl T3):15-27.

5 Heyrman B, De Becker A, Schots R. A case report of immunosuppression-related Kaposi’s sarcoma after autologous stem cell transplantation. BMC Res Notes. 2016;9(1):188. PMid:27012530.

6 Van Leer-Greenberg B, Kole A, Chawla S. Hepatic Kaposi sarcoma: A case report and review of the literature. World J Hepatol. 2017;9(4):171-9. PMid:28217255.

7 Riva G, Luppi M, Barozzi P, Forghieri F, Potenza L. How I treat HHV8/KSHV-related diseases in posttransplant patients. Blood. 2012;120(20):4150-9. PMid:22968461.

8 Lebbe C, Porcher R, Marcelin AG, et al. Human Herpesvirus 8 (HHV8) Transmission and Related Morbidity in Organ Recipients. Am J Transplant. 2013;13(1):207-13. PMid:23057808.

9 Carbone A, Cesarman E, Spina M, Gloghini A, Schulz TF. HIV-associated lymphomas and gamma-herpesviruses. Blood. 2009;113(6):1213-24. PMid:18955561.

10 Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC; 2017.

11 Chadburn A, Said J, Gratzinger D, et al. HHV8/KSHV-Positive Lymphoproliferative disorders and the spectrum of plasmablastic and plasma cell neoplasms: 2015 SH/EAHP Workshop Report-Part 3. Am J Clin Pathol. 2017;147(2):171-87. PMid:28395104.

12 Crane GM, Ambinder RF, Shirley CM, et al. HHV-8 and EBV-positive intravascular lymphoma: An unusual presentation of extracavitary primary effusion lymphoma. Am J Surg Pathol. 2014;38(3):426-32. PMid:24525514.

13 Ferry JA, Sohani AR, Longtine JA, et al. HHV8-positive, EBV-positive Hodgkin lymphoma-like large B-cell lymphoma and HHV8-positive intravascular large B-cell lymphoma. Mod Pathol. 2009;22(5):618-26.

14 Ferreri AJ, Campo E, Seymour JF, et al. Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the ‘cutaneous variant’. Br J Haematol. 2004;127(2):173-83. PMid:15461623.

15 Edelman DC. Human herpesvirus 8- A novel human pathogen. Virol J. 2005;2(1):78. PMid:16138925.

16 Hudnall DS. Crazy 8: Unraveling human herpesvirus 8 seroprevelance. Clin Infect Dis. 2004;39(7):1059-61. PMid:15472861.

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