Does Kaposi's sarcoma predict multicentric Castleman disease in the presence of generalized lymphadenopathy?

Area of Science:

• Oncology
• Virology
• Immunology

Background:

• Multisystemic Castleman disease (MCD) is often linked to HHV8 infection, leading to increased HHV8-positive plasmablasts and potential B-cell lymphoma.
• Previous reports predominantly feature HIV-positive patients coinfected with HHV8, with some developing lymphoma but not Kaposi's sarcoma (KS).

Observation:

• A unique case of an elderly, HIV-negative woman with pre-existing KS presented with symptoms including nausea, vomiting, and fever, alongside diffuse lymphadenopathy.
• Axillary lymph node biopsy revealed HHV8-positive MCD with microlymphoma.

Findings:

• The patient, despite high-dose steroid treatment, experienced multisystem failure and succumbed to the disease.
• This case represents a rare presentation of HHV8+ MCD in an HIV-negative individual with prior KS, distinct from typical HIV-associated MCD.

Implications:

• This case expands the understanding of MCD presentations, particularly in HIV-negative individuals with KS.
• It underscores the aggressive nature of HHV8-associated MCD and its potential to progress rapidly, even without HIV coinfection.
• Further research into the pathogenesis and treatment of MCD in diverse patient populations is warranted.