Hematologic malignancies and hematopoietic stem cell transplantation.

Area of Science:

• Neurology
• Oncology
• Immunology

Background:

• Paraneoplastic neurologic syndromes (PNS) are rare complications of hematologic malignancies.
• Lymphomas are the most frequent hematologic cancers associated with PNS.
• High-risk antibodies are typically absent, except for specific cases like anti-Tr/DNER in cerebellar degeneration.

Approach:

• Review of PNS in hematologic malignancies, focusing on lymphomas and plasma cell disorders.
• Discussion of diagnostic markers like vascular endothelial growth factor (VEGF) in POEMS syndrome.
• Analysis of therapeutic strategies including cancer treatment, immunomodulation, and stem-cell transplantation.

Key Points:

• Peripheral nervous system PNS, particularly demyelinating polyradiculoneuropathy, is associated with non-Hodgkin lymphoma.
• Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome diagnosis relies on criteria and VEGF levels.
• Ophelia syndrome/limbic encephalitis with anti-mGluR5 antibodies shows the best prognosis with potential for complete recovery.

Conclusions:

• Therapeutic approaches for PNS involve cancer treatment and immunomodulatory therapies, with better responses seen in antibody-associated PNS.
• Hematopoietic stem-cell transplantation is a primary treatment for POEMS syndrome, alongside radiotherapy for isolated osteosclerotic lesions.
• Discontinuation of immune checkpoint inhibitors and immunomodulatory treatment are recommended for associated PNS.