HHV8-Positive Multicentric Castleman's Disease and Thrombotic Thrombocytopenic Purpura among HIV-Positive Patients

Roxane Pasquer; Yannick Dieudonné; Pauline Decours; Olivier Hinschberger; Alina Nicolae; Martin Martinot

doi:10.12890/2024_004361

HHV8-Positive Multicentric Castleman's Disease and Thrombotic Thrombocytopenic Purpura among HIV-Positive Patients

Eur J Case Rep Intern Med. 2024 Feb 28;11(3):004361. doi: 10.12890/2024_004361. eCollection 2024.

Authors

Roxane Pasquer¹, Yannick Dieudonné¹, Pauline Decours¹, Olivier Hinschberger², Alina Nicolae³, Martin Martinot⁴

Affiliations

¹ Clinical Immunology Department, Nouvel Hôpital Civil, Strasbourg University, Strasbourg, France.
² Internal Medicine and Clinical Immunology Department, Hôpitaux Civils de Colmar, Colmar, France.
³ Anatomopathology Laboratory, Nouvel Hôpital Civil, Strasbourg, France.
⁴ Infectious Diseases Department, Hôpitaux Civils de Colmar, Colmar, France.

Abstract

Castleman's disease (CD) and thrombotic thrombocytopenic purpura (TTP) are rare diseases that can affect the general population, especially those with HIV. Owing to their rarity, the association between CD and TTP remains insufficiently understood. In this study, we present a case of a 53-year-old patient with controlled HIV infection who presented with fever, lymphadenopathy, severe anaemia, and thrombocytopenia. After a series of tests, the diagnosis was concurrent human herpesvirus 8 (HHV8)-related multicentric CD (MCD) and TTP. Only four male patients were previously reported having this association, with HHV8 present in four and HIV in three patients, suggesting that coinfection with HHV8 and HIV is a pivotal factor in MCD with TTP occurrence.

Learning points: Castleman's disease (CD) and thrombotic thrombocytopenic purpura (TTP) are rare diseases, and their association remains extremely uncommon.We report a case of multicentric CD (MCD) with TTP in a 53-year-old male patient with HIV.Only five patients, including ours, have been reported as having both MCD and TTP, with all five having HHV8 and four having HIV. Thus, coinfection with HHV8 and HIV may be a potential pivotal factor in the occurrence of MCD with TTP.

Keywords: Castleman’s disease; HHV8; HIV; autoimmune hypoglycaemia; thrombotic thrombocytopenic purpura.