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Título

Post-transplant lymphoproliferative disorder myeloradiculopathy in a kidney transplant recipient

RESUMO

Case: 56-year-old Woman presented with weakness in the right leg and urinary retention. She had undergone living-donor kidney transplantation 21 years before for end-stage renal disease, use prednisone and mycophenolate mofetil (MMF). Started right lower back pain and evolved with muscle weakness of the right leg, worse in distal regions, associated with numbness and tingling. In two weeks she was unable to walk and desenvolved urinary retention. Physical examination revealed right lower extremity paresis and reduced sensation to pain and touch below L1 level. Magnetic resonance imaging (MRI) revealed cauda equina enhancement and a nodular formation on left nucleo capsular location. Cerebrospinal fluid (CSF) analysis presented with lynphomonocytic pleocytosis (50 cels), high protein (539 mg/dL) and lactate (82 mg/dL), reduced glucose (9 mg/dl), PCR Epstein-Barr virus (EBV) positive and immunophenotyping with mature clonal B lymphocytes. At this point, we made a diagnosis of Post-transplant lymphoproliferative disorder (PTLD) myeloradiculopathy. MMF was suspended and started chemotherapy (MADIT), Dexamethasone and Rituximab. After four months was observed an important reduction of de nodular lesion and normal CSF. Patient had partial improvement on muscle weakness. Discussion: PTLD is a rare disorder associated to solid organ, stem cell, or bone marrow transplantation. Pathogenesis is presumed to be associated with impairment in the cellular immunity, leading to proliferation of the lymphoid system. EBV is a relevant factor that plays causative and prognostic roles. The disease have a bimodal pattern of incidence, with a peak in the first and a second occurring after 7-10 years. Central nervous system (CNS) PTLD is considered a rare form of the disease, affects mostly supratentorial regions of CNS, specially the basal ganglia, and involvement of the spinal cord is rare. Diagnostic confirmation is performed with biochemical and cytological analysis of blood, CSF and tissue from biopsy (gold standard). Patterns of alterations in MRI may support suspicion. The treatment is focused in reduction of immunosuppression as the first step, radiotherapy and Rituximab with high response and chemotherapy as the standard option. Final considerations: CNS-PTLD representes a potential disabling cause, with specific investigation and treatment. Therefore, it shows the relevance of this discussion in the era of transplantation and immunosuppression.

Palavras Chave

Imunossupression, Myeloradiculophaty, Post-transplant lymphoproliferative disorders

Área

Miscelânea