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- W1991186292 abstract "Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by pathologic inflammation that is almost universally fatal without immune modulating and cytotoxic chemotherapy. Patients with familial HLH or inflammation uncontrolled by medical therapy require stem cell transplant. We reviewed the clinical courses of 26 consecutive patients who underwent first stem cell transplant for HLH to evaluate outcomes and identify clinical factors associated with engraftment and survival. Medical records of all children receiving stem cell transplant for HLH at Texas Children's Hospital (TCH) from 2000-2010 were reviewed. The median time from onset of HLH symptoms to initial chemotherapy was 31 days, with a median time to transplant of 148 days. Many patients had pre-transplant morbidity, with 26% having a Lansky score 80 or less, and 50% demonstrating clinical or lab evidence of inflammation at the start of conditioning. Reduced-intensity conditioning was used for 32% of the transplants. Transplant-related morbidity included high rates of engraftment syndrome (28%), veno-occlusive disease (24%), seizure (16%), acute bleeding (12%), CMV reactivation (48%), EBV reactivation (12%), adenovirus infection (28%), severe mucositis (64%) and acute graft versus host disease (GVHD) (32%). Median time to engraftment was 17.5 days, and there was a high rate of eventual graft failure (21%). Day 100 survival was 81% (95% CI 64-94%), 1 year survival was 61% (95% CI 38-77%), with a longer term survival of 58% (143 days–10 years) and most recent Lansky score > 90 for all survivors. While stem cell transplant is essential for survival in patients with familial and refractory disease HLH, it also results in significant morbidity and mortality. Factors likely include pathogenesis of the disease as well as prolonged prior exposure to cytotoxic chemotherapy and immune suppression. Clinical approaches to improve outcomes should optimize disease control while minimizing toxicity before and after transplant." @default.
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- W1991186292 date "2011-02-01" @default.
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- W1991186292 title "Stem Cell Transplant for HLH: Single Institution Series" @default.
- W1991186292 doi "https://doi.org/10.1016/j.bbmt.2010.12.332" @default.
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