Diplopia as the presenting symptom of a post‐transplant lymphoproliferative disorder in a bilateral inferior lim transplanted young adult

Abstract

Aims/Purpose: To present a unique case of post‐transplant lymphoproliferative disorder (PTLD) after bilateral inferior limb transplantation, which is still controversial due to the absence of precedents in the literature and the unclear risk–benefit ratio.Methods: Case report.Results: A 22‐year‐old male presented to the emergency room complaining of double vision. He had a history of bilateral inferior limb amputation 8 years ago and received a bilateral lower limb transplantation 2 years later. He had normal graft function and was receiving cytomegalovirus prophylaxis. During neurological examinations left abducens nerve (CN VI) palsy was noted. Other cranial nerves affected produced facial hypoesthesia (CN V) and mild paresis (CN VII), hearing loss and tinnitus in left side (CN VIII) and soft palate asymmetry (CN X). He was otherwise neurologically intact, alert and orientated. MRI revealed a lesion located at the facial colliculus at the floor of 4th ventricle. In the context of immunosuppression, the differential diagnosis included abscess versus PTLD. Biopsy of the lesion was performed. Morphologically atypical lymphocytic cells suggestive of B lymphoma were identified. Monoclonal rearrangement for heavy chain Igen and immunoglobulins were identified with molecular biology techniques. The histology features were consistent with a diagnosis of CNS B lineage primary lymphoma. Two days later, bilateral supracondylar amputation was performed. Following the explant, immunosuppression was withdrawn. His underlying condition was finally diagnosed as PTLD, for which he started a chemotherapeutic regimen with radical intention (methotrexate and cytosine arabinoside). The patient recovered fully and up to date, 10 years later, is doing well.Conclusions: Due to its diverse aetiologias, a proper workup must be done of the patients presenting with diplopia to the emergency room. PLTD must be a condition to always be considered in patients with history of solid organ transplant; since the early diagnosis is crucial for a successful management of this often‐fatal condition.