A Single Center Experience of Central Nervous System Manifestations of Graft Versus Host Disease.
Transplantation and Cellular Therapy(2024)
摘要
Background
Central nervous system (CNS) involvement of graft versus host disease (GvHD) is a rare cause of CNS disorders after allogeneic hematopoietic stem cell transplantation (Allo-HSCT). CNS GvHD symptoms are heterogeneous and include cerebrovascular manifestations, demyelinating disease and immune-mediated encephalitis. The 2010 consensus conference on GVHD provided a framework to diagnose this syndrome (1)Cases: We report 8 cases of possible CNS-GVHD from our center. Table 1 summarizes key transplant related characteristics while Table 2 summarizes neurological features of the cases. Altered mental status due to encephalopathy was the most common presentation with a lesser incidence of seizure, hemiparesis, altered higher mental functions as well as cerebellar dysfunction. 6 amongst these had histological evidence of immune-mediated pathological process. Only one case could be diagnosed as definite CNS GvHD as per 2010 guidelines while one more could be diagnosed as probable. Out of the 7 cases who were treated with immunosuppression, 4 showed a partial response. Only two of the cases showed long term survival (>1 year) after the onset of CNS-GvHD.
Conclusions
CNS-related GvHD is a rare cause of CNS disorders after Allo-HSCT and is associated with a poor prognosis. The diagnosis of CNS-GvHD by the 2010 consensus criteria is based on rigid criteria and does not cover a majority of cases with strong clinical suspicion.
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