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[Peripheral neuropathy in large granular lymphocytic leukemia]
No to Shinkei = Brain and Nerve
|August 1, 1993
Summary
A teen with LGL leukemia developed peripheral neuropathy and virus-associated hemophagocytic syndrome (VAHS)-like symptoms. Treatment with methyl-prednisolone and immunoglobulin therapy improved neurological findings, suggesting macrophage activation in neuropathy.
Area of Science:
- Neurology
- Hematology
- Immunology
Background:
- Large granular lymphocyte (LGL) leukemia can present with autoimmune phenomena.
- Virus-associated hemophagocytic syndrome (VAHS) is a severe, life-threatening condition.
- Epstein-Barr virus (EBV) infection can trigger immune dysregulation.
Observation:
- A 16-year-old female with LGL leukemia exhibited VAHS-like symptoms: fever, liver dysfunction, splenomegaly, hepatomegaly, and pancytopenia.
- The patient presented with peripheral neuropathy, including dysesthesia, paresthesia, swelling, and tenderness in lower extremities, progressing to weakness, atrophy, and areflexia.
- Cerebrospinal fluid analysis revealed pleocytosis and elevated protein levels, indicating central nervous system inflammation.
Findings:
- Chronic active Epstein-Barr virus (EBV) infection was confirmed by specific antibody titers.
- Elevated neopterin levels in cerebrospinal fluid suggested activated macrophages as a cause of peripheral neuropathy.
- Neurological symptoms showed significant improvement following pulse methyl-prednisolone and high-dose intravenous immunoglobulin therapy.
Implications:
- This case highlights a rare presentation of LGL leukemia associated with EBV-driven VAHS and peripheral neuropathy.
- Activated macrophages, indicated by elevated CSF neopterin, may play a crucial role in the pathogenesis of EBV-associated neuropathy.
- Effective management involves addressing both the underlying leukemia and the immune-mediated complications, including prompt immunosuppressive and immunoglobulin therapy.