Oral Kaposi Sarcoma and Multicentric Castleman Disease in Advanced AIDS
- A rare medical case involving a 35-year-old male patient highlights the clinical overlap of Kaposi sarcoma and multicentric Castleman disease in an individual with advanced AIDS, according to...
- To investigate the widespread lymphadenopathy and systemic symptoms, clinicians performed an excisional biopsy of the left supraclavicular lymph node.
- Castleman disease comprises a group of rare lymphoproliferative disorders with clinical manifestations ranging from asymptomatic presentation to widespread lymphadenopathy with systemic involvement.
A rare medical case involving a 35-year-old male patient highlights the clinical overlap of Kaposi sarcoma and multicentric Castleman disease in an individual with advanced AIDS, according to findings detailed in Cureus. The patient presented with severe back pain persisting for one week, alongside a prior acquired immune deficiency syndrome diagnosis spanning 12 years and existing Kaposi sarcoma while undergoing highly active antiretroviral therapy.
Radiographic imaging performed during the clinical evaluation revealed hepatomegaly and diffuse lymphadenopathy. Laboratory tests showed an HIV viral load below 20 polymerase chain reaction copies per milliliter, an absolute CD4 count of 453 cells per microliter, and a CD8 count of 4,142 cells per microliter.
Diagnosis and Biopsy Findings
To investigate the widespread lymphadenopathy and systemic symptoms, clinicians performed an excisional biopsy of the left supraclavicular lymph node. The pathological evaluation identified human herpesvirus-8-positive Kaposi sarcoma occurring in the background of the plasmacytic variant of multicentric Castleman disease.
According to the case documentation, the examination showed no evidence of transformation into large B-cell or plasmablastic lymphoma at the time of the biopsy. The patient was discharged on highly active antiretroviral therapy with subsequent follow-up arranged for a chemotherapy regimen consisting of cyclophosphamide, adriamycin, vincristine, and prednisone, combined with rituximab and prophylaxis for pneumocystis carinii.
Medical Context of Multicentric Castleman Disease
Castleman disease comprises a group of rare lymphoproliferative disorders with clinical manifestations ranging from asymptomatic presentation to widespread lymphadenopathy with systemic involvement. Cases are classified as either localized unicentric disease or generalized multicentric disease, and further divided into hyaline vascular, plasma cell, or mixed variants based on histological findings.
The plasma cell variant accounts for 10% to 20% of cases and involves constitutional symptoms alongside enlarged lymph nodes, while the hyaline vascular variant represents approximately 70% of cases. A less common subvariant of the plasma cell type features large plasmablasts harboring human herpesvirus-8 with the potential to progress to plasmablastic monoclonal lymphoma.
Plasmablastic multicentric Castleman disease is aggressive, multifocal, and frequently diagnosed in individuals with HIV infections. The report notes that the condition has gained clinical relevance due to its association with HIV and human herpesvirus-8, as well as its risk of progression to plasmablastic B-cell lymphoma, which carries a high mortality rate despite treatment.
