Title : Human herpesvirus-8 negative in patient with non multicentric castleman disease as the first manifestation of HIV disease
Background: Patients with acquired immunodeficiency syndrome HIV disease can present with a constellation of symptoms and syndromes. In this abstract we describe a rare case of human herpesvirus 8 (HHV-8) apparently unrelated and not multicentric Castleman disease (CD), as the presenting manifestation of HIV disease. In the context of HIV infection CD is well described and it is almost always multicentric and linked to HHV-8. There are limited published data surrounding HHV-8–unrelated CD among HIV-positive and -negative patients.
Case: We present a case of young 24-year-old boy infected with HIV at first detection with multiple lymphadenopathy whose histology describes a Castelman like picture with negative HHV 8 serology. We have come to our attention this young Brazilian born but adopted by an Italian family at the age of about 6 years. At the onset for which he presented to the ED was fever, asthenia and multiple lymphadenopathies. Laboratory tests: initial lymphopenia, inflammation indices poorly represented, serology for major viruses (CMV-EBV, HBV-HCV-syphilis-quantiferon-toxoplasmaparvovirus) negative. Abdominal lymphadenopathy and splenomegaly were present on the CT scan of the abdomen. Leishmania is also positive for serology and IL-6 2.7pg/mL. A positive HIV test was performed to complete the serum-virological picture. Furthermore, the values of CD4 193/mmc and HIV-RNA 4.730.500 cp/ml are reported, the genotyping test showed a pharmacological pattern of totisensitivity and the avidity for HIV was high precisely to document an acquisition of the disease for several months. He underwent lymph node exeresis whose histology described a picture compatible with Castelman-like. HHV 8 serology was negative. The patient was started on antiretroviral therapy and for the moment on haematological monitoring.
Conclusion: A subset of multicentric CD (MCD) is caused by human herpesvirus-8,whereas HHV- 8–negative MCD cases remain idiopathic (iMCD). Unicentric CD (UCD) involves a single lymph node region showing characteristic “Castleman-like” histopathologic changes. Inflammatory manifestations are generally mild in UCD and usually disappear after surgical excision of the lymph node. HHV-8–associated MCD is most commonly diagnosed in HIV infected or otherwise immunocompromised individuals. However, >50% of MCD cases are HIV and HHV-8 negative.The etiology of iMCD is unknown, although it is hypothesized to involve one or more of the following mechanisms: autoimmunity/autoinflammation, paraneoplastic or infection with a virus other than HHV-8.