Case Presentation: A 44-year-old Ugandan man with a history of HIV/AIDS on Bictegravir-Emtricitabine-Tenofovir Alafenamide presented with fevers and abdominal pain. Past medical history was significant for HIV/AIDS previously complicated by CNS Toxoplasmosis. Physical exam was notable for diaphoresis, diffuse lymphadenopathy, and abdominal distension. Laboratory values were significant for viral load 520 copies/mL and CD4 count 133/uL. CT abdomen and pelvis showed splenomegaly and extensive lymphadenopathy. He was started on broad spectrum antibiotics; however, blood cultures were negative after five days and antibiotics were discontinued. A broad infectious work up was negative. The patient underwent excisional lymph node biopsy with subsequent negative cultures. Lymph node pathology was consistent with Kaposi’s sarcoma (KS) and HHV8-positive multicentric Castleman’s disease (KSHV-MCD). Oncology was consulted and the patient received one dose of Liposomal Doxorubicin prior to discharge with a plan to continue outpatient treatment. A decision to add Rituximab to the treatment regimen was deferred at the time of discharge given positive Hepatitis B serologies. The patient continued ART on discharge. Appropriate follow up was arranged.
Discussion: Incidence of KS increased with the onset of the AIDS epidemic in the 1980s. By 1987, the development of HAART resulted in improved host immunity and decreased risk of KS. KS is characterized by disseminated cutaneous disease, which often involves the oral mucosa and viscera. Dermatologic manifestations include purplish or brown macules, plaques or nodules that are prone to ulceration or bleeding. Diagnosis of KS is based on histological findings. The basis of treatment is HAART. The first line agent for systematic treatment of KS is liposomal doxorubicin. KSHV-MCD is a B cell lymphoproliferative disease that typically develops in HIV-infected patients despite preserved CD4 counts. KSHV encodes a viral homology of cellular IL-6 that plays a role in the pathogenesis of KSHV-MCD and contributes to the inflammatory symptoms seen with disease. Clinical manifestations include generalized lymphadenopathy, splenomegaly, fevers, weight loss, and edema. Lymph node biopsy is required to make a diagnosis of KSHV-MCD. Rituximab is an effective single agent chemotherapy for treatment of KSHV-MCD.Previous cases of combined KSHV-MCD and KS are reported, however the mechanism of simultaneous presence is unknown. In KS, KSHV is found in its latent phase. In contrast, in KSHV-MCD, KSHV is in its lytically active form. Symptomatic flares of KSHV-MCD are thought to be related to high KSHV-viral loads. We suspect our patient’s presentation was due to an inflammatory syndrome due to a high lytically active KSHV viral load.
Conclusions: This case highlights the importance of considering KS or KSHV-MCD as alternative diagnoses in patients with HIV presenting with unexplained presumed sepsis and lymphadenopathy.