HLH Clinical Trial
Official title:
Teniposide Incorporating Bu/Cy Conditioning Regimen Followed by HSCT for HLH With Central Nervous System Involvement: a Prospective Single-arm Clinical Study
Hemophagocytic lymphohistiocytosis (HLH) is a highly inflammatory disease involving cytokine storms and is characterised by rapid progression and high mortality. There are no uniform clinical criteria for the diagnosis of CNS-HLH.And there is still a lack of international consensus on the treatment for CNS-HLH. Following allogeneic HSCT after HLH induction therapy is recommended for CNS-HLH. One of the major factors influencing the transplantation effect is conditioning. Therefore, we conduct a prospective clinical study to explore the efficacy and safety of Teniposide incorporating Bu/Cy conditioning regimen followed by allogeneic hematopoietic stem cell transplantation for HLH with central nervous system involvement.
Hemophagocytic lymphohistiocytosis (HLH) is a highly inflammatory disease involving cytokine storms and is characterised by rapid progression and high mortality. HLH with central nervous system involvement (CNS-HLH) is a type of inflammatory factor storm that affects the blood-brain barrier and further infiltrates brain tissue, causing patients to exhibit corresponding neurological and/or psychiatric symptoms. It can be the first symptom of HLH or occur in the later stages of HLH, and can be an independent symptom or accompanied by other symptoms. In recent years, with the deepening of research on HLH, CNS-HLH has gradually received attention and is often associated with poor prognosis. There are no uniform clinical criteria for the diagnosis of CNS-HLH. And there is still a lack of international consensus on the treatment for CNS-HLH. Following allogeneic HSCT after HLH induction therapy is recommended for CNS-HLH. One of the major factors influencing the transplantation effect is conditioning. Therefore, we conduct a prospective clinical study to explore the efficacy and safety of Teniposide incorporating Bu/Cy conditioning regimen followed by allogeneic hematopoietic stem cell transplantation for CNS-HLH. ;
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