An Immunocompetent HIV-Negative Elderly Patient with Low-Grade Fever, Generalized Lymphadenopathy, Splenomegaly, and Acute Phase Response: Do Not Forget Castleman Disease.
Kalliopi AzariadisMaria IoannouKalliopi ZachouGeorgios N DalekosPublished in: Case reports in infectious diseases (2021)
Multicentric Castleman disease (MCD) is a rare lymphoproliferative disorder that mainly affects middle-aged patients with human immunodeficiency virus (HIV) infection. However, HIV-negative patients can also be affected representing a small proportion of the total MCD cases. Of note, recent studies from China in HIV-negative patients with MCD have suggested that the onset of the disease can be observed in younger age than previously thought. If undiagnosed and untreated, the MCD has a poor prognosis and may progress to lymphoma. We present an 82-year-old immunocompetent male patient who was admitted to our department because of low-grade fever, cachexia, anasarca, hepatosplenomegaly, and generalized lymphadenopathy. Laboratory findings showed anemia and increased markers of inflammation including hyperferritinemia and polyclonal hyperglobulinemia. Infectious causes including HIV were ruled out. Histological examination of a cervical lymph-node revealed lesions supportive of MCD diagnosis. Of note, the outer-zone plasmablasts' nuclei stained positive for human herpesvirus-8 (HHV8). The patient received 4 cycles of cyclophosphamide, vincristine, and dexamethasone with regression of all symptoms. This case underlines that HHV8-associated MCD should be considered as a rare cause of generalized lymphadenopathy even in HIV-negative immunocompetent patients when other causes have been appropriately excluded because a timely diagnosis can be life-saving.
Keyphrases
- human immunodeficiency virus
- antiretroviral therapy
- low grade
- hiv infected
- hiv positive
- hepatitis c virus
- hiv aids
- hiv testing
- poor prognosis
- end stage renal disease
- middle aged
- chronic kidney disease
- high grade
- men who have sex with men
- lymph node
- newly diagnosed
- ejection fraction
- case report
- prognostic factors
- endothelial cells
- long non coding rna
- epstein barr virus
- oxidative stress
- early stage