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Comparison of hematopoietic cell transplant conditioning regimens for hemophagocytic lymphohistiocytosis disorders
- Source :
- The Journal of allergy and clinical immunology, vol 149, iss 3, J Allergy Clin Immunol
- Publication Year :
- 2022
- Publisher :
- Elsevier BV, 2022.
-
Abstract
- BackgroundAllogeneic hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis (HLH) disorders is associated with substantial morbidity and mortality.ObjectiveThe effect of conditioning regimen groups of varying intensity on outcomes after transplantation was examined to identify an optimal regimen or regimens for HLH disorders.MethodsWe studied 261 patients with HLH disorders transplanted between 2005 and 2018. Risk factors for transplantation outcomes by conditioning regimen groups were studied by Cox regression models.ResultsFour regimen groups were studied: (1) fludarabine (Flu) and melphalan (Mel) in 123 subjects; (2) Flu, Mel, and thiotepa (TT) in 28 subjects; (3) Flu and busulfan (Bu) in 14 subjects; and (4) Bu and cyclophosphamide (Cy) in 96 subjects. The day 100 incidence of veno-occlusive disease was lower with Flu/Mel (4%) and Flu/Mel/TT (0%) compared to Flu/Bu (14%) and Bu/Cy (22%) (P< .001). The 6-month incidence of viral infections was highest after Flu/Mel (72%) and Flu/Mel/TT (64%) compared to Flu/Bu (39%) and Bu/Cy (38%) (P< .001). Five-year event-free survival (alive and engrafted without additional cell product administration) was lower with Flu/Mel (44%) compared to Flu/Mel/TT (70%), Flu/Bu (79%), and Bu/Cy (61%) (P= .002). The corresponding 5-year overall survival values were 68%, 75%, 86%, and 64%, and did not differ by conditioning regimen (P= .19). Low event-free survival with Flu/Mel is attributed to high graft failure (42%) compared to Flu/Mel/TT (15%), Flu/Bu (7%), and Bu/Cy (18%) (P< .001).ConclusionsGiven the high rate of graft failure with Flu/Mel and the high rate of veno-occlusive disease with Bu/Cy and Flu/Bu, Flu/Mel/TT may be preferred for HLH disorders. Prospective studies are warranted.
- Subjects :
- Melphalan
medicine.medical_specialty
Transplantation Conditioning
Allergy
bone marrow transplantation
medicine.medical_treatment
Hemophagocytic
Immunology
Graft vs Host Disease
Hemophagocytic lymphohistiocytosis
Hematopoietic stem cell transplantation
ThioTEPA
Gastroenterology
Article
Lymphohistiocytosis, Hemophagocytic
hemic and lymphatic diseases
Internal medicine
medicine
Humans
Immunology and Allergy
Busulfan
Cyclophosphamide
neoplasms
Lymphohistiocytosis
Transplantation
allogeneic hematopoietic cell transplantation
BMT
business.industry
Prevention
Hematopoietic Stem Cell Transplantation
virus diseases
Hematology
medicine.disease
respiratory tract diseases
Fludarabine
Regimen
Infectious Diseases
Good Health and Well Being
HCT
HSCT
business
Thiotepa
Vidarabine
HLH
medicine.drug
Subjects
Details
- ISSN :
- 00916749
- Volume :
- 149
- Database :
- OpenAIRE
- Journal :
- Journal of Allergy and Clinical Immunology
- Accession number :
- edsair.doi.dedup.....036dcf29539e7f11c3dc06d93091758a