[Crow-Fukase (POEMS) syndrome]

Area of Science:

• Neurology
• Hematology
• Immunology

Background:

• Crow-Fukase syndrome, or POEMS syndrome, is a rare multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes.
• Its pathogenesis is complex, with vascular endothelial growth factor (VEGF) overproduction by monoclonal plasma cells implicated in many symptoms, alongside other upregulated cytokines.
• The precise mechanisms leading to peripheral neuropathy, including VEGF's role in blood-nerve barrier disruption and microangiopathy, are still under investigation.

Purpose of the Study:

• To elucidate the pathogenesis and potential therapeutic strategies for Crow-Fukase (POEMS) syndrome.
• To highlight the role of VEGF and monoclonal plasma cell proliferation in the syndrome's development.
• To discuss current and potential future treatment options, including stem cell transplantation and targeted therapies.

Main Methods:

• Review of existing literature on Crow-Fukase (POEMS) syndrome pathogenesis and treatment.
• Analysis of molecular findings, such as V(lambda) gene usage in M-protein.
• Evaluation of treatment outcomes, particularly high-dose chemotherapy with autologous stem cell transplantation and novel agents.

Main Results:

• VEGF overproduction is a key factor, potentially affecting the blood-nerve barrier and contributing to neuropathy.
• Molecular studies suggest specific V(lambda) gene patterns are associated with the syndrome.
• High-dose chemotherapy with autologous peripheral blood stem cell transplantation demonstrates significant improvement in neuropathy and other symptoms, with decreased VEGF levels.

Conclusions:

• Crow-Fukase (POEMS) syndrome is a complex disorder with VEGF playing a central pathogenic role.
• High-dose chemotherapy and stem cell transplantation offer a promising treatment option for eligible patients.
• Future therapies may involve thalidomide, lenalidomide, or anti-VEGF antibodies like bevacizumab.