Case Presentation: A 42-year-old male with HIV (CD4 79, Viral Load >1.35 Million) presented to the hospital with altered mental status, fever, tachycardia, axillary lymphadenopathy and cachexia. Initial work-up revealed profound anemia with a hemoglobin of 3.0 and no identifiable source of bleeding. Infectious screen was positive for norovirus and Enteropathogenic E. coli gastroenteritis, and serology was positive for autoimmune hemolytic anemia. The patient was promptly started on high-dose prednisone, empiric antibiotic treatment, Biktarvy, and Atovaquone. Pertinent labs included an elevated ESR and CRP, SPEP with immunofixation showed a polyclonal gamma elevation, IL-6 (62.4), VEGF (171), HHV8 PCR Quant: 4.5 E+07, IgM (558) and IgG (2141). CT imaging revealed diffuse abdominal and axillary lymphadenopathy, prompting biopsy, which confirmed Kaposi’s Sarcoma-associated Herpesvirus HHV8 and multicentric Castleman’s disease (KSHV-MCD). The patient subsequently developed septic shock and acute respiratory failure, requiring intubation and continuous renal replacement therapy. Infectious disease consultants recommended continuation of empiric antibiotics, while hematology advised withholding chemotherapy due to concerns for immune reconstitution inflammatory syndrome. The patient was treated with Solumedrol, Ganciclovir, and Rituximab. His course was complicated by fungemia, bacteremia, and high-grade AV block requiring transvenous pacemaker placement. He continued to experience refractory multiorgan failure and was eventually transitioned to comfort care.
Discussion: KSHV-MCD is a systemic inflammatory disorder characterized by lymphadenopathy, elevated inflammatory markers (e.g., ESR, CRP, IL-6), and immune dysregulation. MCD consists of two forms: an idiopathic form, which is rare and seen in immunocompetent individuals, and an HIV-associated form, which is driven by HHV8 and presents aggressively. Patients with HHV8-associated MCD often develop Kaposi’s sarcoma (KS), a malignant neoplasm of endothelial cells. The presentation of KSHV-MCD includes constitutional symptoms and lymphoproliferation. As seen in this case, anemia and multi-organ dysfunction may also occur due to systemic inflammation and bone marrow suppression. Diagnosis involves imaging to assess lymphadenopathy, biopsy for histopathological confirmation, and viral PCR to detect HHV8. Management of KSHV-MCD is multifaceted, focusing on HIV control and suppression of the HHV8 infection and inflammation. Antiretroviral therapy remains the cornerstone of treatment. Immunosuppressive therapies, particularly corticosteroids, are used to address the inflammatory components of MCD. Rituximab, a monoclonal antibody targeting B-cells, is often used alongside IL-6 Inhibitors. The prognosis for KSHV-MCD remains poor, particularly when complicated by multi-organ failure.
Conclusions: This case highlights the severe and often fatal course of KSHV-MCD. Despite advances in HIV treatment, the prognosis remains challenging due to the aggressive nature of the disease and advanced immunosuppression, necessitating prompt diagnosis and treatment.