A Single Center Experience of Central Nervous System Manifestations of Graft Versus Host Disease.

Transplantation and Cellular Therapy(2024)

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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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