Spontaneous Regression Of Central Nervous System Posttransplant Lymphoproliferative Disease: A Case Report
Gao, R., Zhang, Y., Chen, G., Bhekharee, A. K., Du, Z., & Chu, S. (2021). Spontaneous regression of central nervous system posttransplant lymphoproliferative disease: A case report. Medicine, 100(6), e24713. https://doi.org/10.1097/MD.0000000000024713
View Original Source →Abstract
RATIONALE: 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. PATIENT CONCERNS: 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. DIAGNOSES: 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. INTERVENTIONS: She was referred to hematology department to receive rituximab. OUTCOMES: After 4 rounds of treatment, the lesion resolved satisfactorily. LESSONS: 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 at 23 years of age because of renal failure caused by IGA nephropathy. At age 36, brain biopsy revealed lymphoid hyperplasia
Clinical Characteristics
1-month-long progressive right-sided visual field defect. Lumbar puncture revealed elevated opening pressure, 211 sequence of ebv were found in cerebrospinal fluid (csf). Brain MRI demonstrated a rim-enhanced mass in the left temporal lobe. Posttransplant lymphoproliferative disease (ptld) was suspected. A second brain biopsy was performed. Pathology still suggested p-ptld. She was referred to hematology department and received 500 mg rituximab injection every month for 4months
Remission Characteristics
Her vision field gradually recovered and brain MRI showed significant improvement
Treatment & Mechanisms
Proposed Remission Mechanisms
The mechanism behind self-remission is significant antigenicity conferred by ebv viral sequences incorporated within tumor genome.
Clinical Treatment
Biopsy rituximab
Non-Clinical Treatment
None reported