Spontaneous Regression Of Central Nervous System Posttransplant Lymphoproliferative Disease
Gao R, Zhang Y, Chen G, Bhekharee AK, Du Z, Chu S. Spontaneous regression of central nervous system posttransplant lymphoproliferative disease: A case report. Medicine (Baltimore). 2021 Feb 12;100(6):e24713. doi: 10.1097/MD.0000000000024713. PMID: 33578612; PMCID: PMC10545224.
View Original Source →Abstract
Primary central nervous system (CNS) posttransplant lymphoproliferative disease (PTLD) is a very rare entity. Patients may respond to reduction of immunosuppression or other therapies, but the prognosis is still pessimistic. Herein, we report a 40-year-old female with a history of renal transplantation developed brain masses 4 years ago. Although brain biopsy was performed, PTLD was underdiagnosed then. No relevant treatment was administered. However, the lesions resolved spontaneously. After 4 years, new lesion appeared in a different brain region. The history of renal transplantation raised the suspicion of PTLD. Reexamination of previous brain sections confirmed the diagnosis of polymorphic PTLD (P-PTLD). A second biopsy of the new lesion also demonstrated P-PTLD. She was referred to hematology department to receive rituximab. After 4 rounds of treatment, the lesion resolved satisfactorily. This case demonstrates the natural history of primary CNS P-PTLD. Although self-remission and recurrence is possible, aggressive measures should be taken to this condition.
Case Details
Disease Location
Brain
Personal Characteristics
40-year-old female, kidney transplant 17 years ago because of renal failure caused by IGA nephropathy, four years ago, she developed double vision and left-sided weakness. Brain MRI revealed right thalamus and midbrain rim-enhanced lesions. Brain biopsy revealed lymphoid hyperplasia.
Clinical Characteristics
1-month-long progressive right-sided visual field defect. On admission, neurological examinations found a right visual field defect. Positive ebv DNA in csf. Brain MRI demonstrated a rim-enhanced mass in the left temporal lobe. A second brain biopsy was performed. Pathology still suggested p-ptld
Remission Characteristics
Her vision field gradually recovered and brain MRI showed significant improvement
Treatment & Mechanisms
Clinical Treatment
Biopsy 500 mg rituximab injection every month for 4months