Abstract
In 1992, Kaposi sarcoma herpesvirus (KSHV/HHV8) was discovered and identified as the causative agent for Kaposi sarcoma. Subsequently, the presence of this virus has been detected in a number of lymphoproliferative disorders in people living with HIV (PLWH), including: KSHV-associated multicentric Castleman disease, primary effusion lymphoma, KSHV-positive diffuse large B-cell lymphoma, and germinotropic lymphoproliferative disorder. Each of these rare entities has subsequently been diagnosed in HIV-negative individuals. The recognition of some of these KSHV/HHV8-associated lymphoproliferative disorders has led to their inclusion in the WHO classification of lymphomas in 2008 and the revision of 2016; however, further revision is under way to update the classification. The relatively recent recognition of these lymphoproliferative disorders and their low incidence, particularly in the HIV-negative population, means that there is little published evidence and consensus on their clinical features and management. The publication of a new WHO classification of lymphomas should yield diagnostic clarity, providing an impetus for retrospective case series and prospective clinical trials in these KSHV/HHV8-associated lymphoproliferative disorders.
Highlights
On 5 June 1981, a report in the Mortality Morbidity Weekly Report (MMWR) of clusters of Pneumocystis pneumonia heralded the AIDS epidemic
It subsequently became apparent that the incidence of Kaposi sarcoma (KS) was increased many thousands of times amongst people living with HIV (PLWH)
In 1994, the novel oncogenic herpes virus known as Kaposi sarcoma herpesvirus (KSHV), or Human herpesvirus 8 (HHV8), was discovered by Yuan Chang and her husband Patrick Moore [2]
Summary
On 5 June 1981, a report in the Mortality Morbidity Weekly Report (MMWR) of clusters of Pneumocystis pneumonia heralded the AIDS epidemic. KSHV/HHV8 is detectable in the malignant spindle cells of all forms of KS, whether associated with HIV infection, allograft recipients, or classical or endemic forms of KS. In 1995, the presence of KSHV/HHV8 was detected in a form of plasmablastic multicentric Castleman disease, seen most frequently in PLWH [5]. Recent iterations of the WHO classification of haematological malignancy have included KSHV/HHV8-associated diffuse large B-cell lymphomas, which have frequently arisen on a background of KSHV/HHV8-associated multicentric Castleman disease. Whilst most KSHV/HHV8-associated lymphoproliferative disorders occur more frequently in PLWH, solid organ allograft recipients, and those from KSHV/HHV8 endemic areas, GLPD is most commonly seen in immunocompetent individuals. Based on the knowledge of these entities, the WHO classification of lymphomas considered the immunodeficiency-related lymphoproliferations and included the spectrum of KSHV/HHV8- and EBV-related disorders in PLWH, as well as in HIV-negative individuals
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