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A case of lymphomatoid granulomatosis with central nervous system involvement successfully treated with IFNα
- Source :
- International journal of hematology. 114(4)
- Publication Year :
- 2021
-
Abstract
- Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV)-driven B-cell lymphoproliferative disease affecting mainly extranodal sites such as the lung, central nervous system (CNS), skin, kidney, and liver. We report a case of low-grade LYG involving the CNS that was successfully treated with interferon alpha (IFNα). A 69-year-old woman developed necrotic erythema of the skin and was initially diagnosed with pyoderma gangrenosum based on skin biopsy. She showed a limited response to prednisolone. Approximately 6 months after the initial onset, low-grade LYG was diagnosed after detection of CNS lesions on brain biopsy. The whole blood EBV-DNA load determined by real-time polymerase chain reaction was slightly elevated. Two months into IFNα therapy, skin and CNS lesions had responded favorably and the EBV-DNA load decreased. IFNα plays an important role in treatment of LYG through its antiproliferative, immunomodulatory, and anti-EBV effects. To our knowledge, this is the first case report of successful treatment with IFNα in Japan. Further investigation is necessary to determine optimal use of IFNα for LYG.
- Subjects :
- Pathology
medicine.medical_specialty
Lymphomatoid granulomatosis
Erythema
Biopsy
Alpha interferon
Central Nervous System Neoplasms
Clonal Evolution
03 medical and health sciences
0302 clinical medicine
Internal medicine
medicine
Humans
Aged
Hematology
medicine.diagnostic_test
business.industry
Brain biopsy
Interferon-alpha
Lymphomatoid Granulomatosis
medicine.disease
Immunohistochemistry
Magnetic Resonance Imaging
Treatment Outcome
030220 oncology & carcinogenesis
Skin biopsy
Prednisolone
Female
medicine.symptom
Symptom Assessment
business
Tomography, X-Ray Computed
Pyoderma gangrenosum
Biomarkers
030215 immunology
medicine.drug
Subjects
Details
- ISSN :
- 18653774
- Volume :
- 114
- Issue :
- 4
- Database :
- OpenAIRE
- Journal :
- International journal of hematology
- Accession number :
- edsair.doi.dedup.....ae0b2d8747be47df14e1b145cec76463