Primary cutaneous diffuse large B-cell lymphoma, leg type, with features simulating POEMS syndrome

Area of Science:

• Oncology
• Hematology
• Dermatology

Background:

• Primary cutaneous diffuse large B-cell lymphoma (PCLBCL), leg type, is a rare non-Hodgkin lymphoma.
• POEMS syndrome is a multisystem disorder typically associated with a monoclonal plasma cell proliferative disorder.

Observation:

• A 91-year-old woman presented with a leg PCLBCL and POEMS-like symptoms (hepatomegaly, endocrinopathy, edema, skin changes, polyneuropathy) but lacked a monoclonal plasma cell disorder.
• Elevated serum vascular endothelial growth factor (VEGF) and interleukin-6 (IL-6) were observed.
• Lymphoma cells showed positive immunostaining for VEGF and IL-6.

Findings:

• This is the first reported case of PCLBCL, leg type, associated with POEMS-like syndrome.
• The study suggests that cytokines (VEGF, IL-6) secreted by lymphoma cells may induce POEMS-like symptoms.
• The findings challenge the necessity of a monoclonal plasma cell disorder for POEMS syndrome diagnosis.

Implications:

• This case expands the understanding of PCLBCL and its potential systemic manifestations.
• It suggests reconsidering diagnostic criteria for POEMS syndrome to include cytokine-driven presentations.
• Further research is warranted to explore the role of cytokines in POEMS-like syndromes and PCLBCL.