Isolated Cervical Spinal Cord Post-transplant Lymphoproliferative Disorder Presenting With Acute Myelopathy
Prach Uthayo1
1Neurology, Police General Hospital
Objective:
Not applicable
Background:
Post-transplant lymphoproliferative disorder (PTLD) represents a spectrum of lymphoid proliferations occurring after solid organ or hematopoietic stem cell transplantation under chronic immunosuppression. Central nervous system (CNS) involvement is uncommon, accounting for 5–30% of PTLD cases, and isolated spinal cord disease is exceptionally rare. Early recognition is critical, as it often mimics infection or primary CNS malignancy, leading to diagnostic delay.
Design/Methods:
Not applicable
Results:

A man in his early 50s with focal segmental glomerulosclerosis and kidney transplantation 10 years earlier presented with progressive numbness and weakness of the right arm. Neurological examination showed decreased pinprick sensation in C6–C8 dermatomes and mild weakness in corresponding myotomes. Cervical spine MRI revealed a 1.1 × 0.5 × 0.7 cm rim-enhancing intramedullary lesion at C1 with restricted diffusion and surrounding edema extending to C3. The leading provisional diagnosis was CNS-PTLD, although pyogenic abscess, metastasis, and glioblastoma were also considered.

Because of the lesion’s high-risk intramedullary location, biopsy was initially deferred after multidisciplinary discussion. The patient received intravenous dexamethasone 4 mg every 6 hours, ceftriaxone, and ampicillin, with reduced immunosuppression. MRI initially showed partial improvement, but symptoms and lesion size worsened after steroid taper. A subsequent C1–C2 biopsy confirmed CD20-positive, EBER-negative lymphoid cells consistent with PTLD. The patient commenced R-CHOP chemotherapy but developed Chryseobacterium indologenes septic shock and multiorgan failure, leading to death two weeks later.

Conclusions:

This case highlights an extremely rare presentation of CNS-PTLD as an isolated intramedullary cervical spinal cord mass. To our knowledge, this is the first reported case of PTLD presenting solely with an isolated intramedullary spinal cord lesion. It emphasizes the importance of considering PTLD in the differential diagnosis of spinal cord lesions in transplant recipients.

Disclosure:
This case report has been submitted for publication in BMJ Case Reports and is currently under review and waiting for publication.

10.1212/WNL.0000000000215381
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