scholarly journals Correlation analysis of severe aplastic anemia immunosuppressive therapy and human leukocyte antigen alleles in pediatric patients

2015 ◽  
Vol 10 (6) ◽  
pp. 2396-2402
Author(s):  
RU-TING FU ◽  
HONG-MAN XUE ◽  
BI-HONG ZHANG ◽  
JIAN WANG ◽  
SHAO-FEN LIN ◽  
...  
Keyword(s):  
Correlation Analysis ◽  
Human Leukocyte Antigen ◽  
Aplastic Anemia ◽  
Immunosuppressive Therapy ◽  
Pediatric Patients ◽  
Human Leukocyte ◽  
Severe Aplastic Anemia ◽  
Leukocyte Antigen

Expression of Human Leukocyte Antigen B61 Is Associated with Idiopathic Aplastic Anemia, Hepatitis-Associated Aplastic Anemia and Fulminant Hepatic Failure in Children

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3487-3487 ◽  
Author(s):  
Yoshiyuki Takahashi ◽  
Akira Ohara ◽  
Ryoji Kobayashi ◽  
Hiromasa Yabe ◽  
Akira Kikuchi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Liver Transplantation ◽  
Human Leukocyte Antigen ◽  
Aplastic Anemia ◽  
Immunosuppressive Therapy ◽  
Fulminant Hepatic Failure ◽  
Hepatic Failure ◽  
Human Leukocyte ◽  
Healthy Controls ◽  
Leukocyte Antigen

Abstract Abstract 3487 Introduction: Increasing evidence supports the notion that aplastic anemia (AA) defined as peripheral blood pancytopenia and bone marrow hypoplasia is an autoimmune disease mediated by T-cells attacking hematopoietic progenitor cells. Around 10% of acquired AA is associated with acute hepatitis, yet a causative virus has not been identified in most patients. Like other autoimmune disorders, adult AA is associated with human leukocyte antigen (HLA)-DR15. However, HLA-DR15 is not significantly associated in children with AA (Fuhrer et al. Pediatric Blood Cancer 2007 and Yoshida et al. Br J Haematol 2008). Human leukocyte antigen associations might differ between children and adults with AA. We therefore investigated the HLA types among patients with idiopathic aplastic anemia, hepatitis-associated aplastic anemia and fulminant hepatic failure who underwent liver transplantation. Patients and methods: We investigated data from 321 children (male, n = 202; female, n = 119; median age, 10 [range 0 – 18] years) with severe and very severe AA. All patients were enrolled in the AA-92 or AA-97 study from 1992 to 2010 and received immunosuppressive therapy (IST) using antithymocyte globulin and cyclosporine. Two hundred and sixty nine children had idiopathic AA and 52 had AA associated with hepatitis. The HLA alleles of 25 children with fulminant hepatic failure who underwent liver transplantation between 2006 and 2011 were characterized. Differences in all HLA alleles between the patients and healthy controls in the Japanese population were statistically analyzed at the phenotype level by χ2 and t tests using PRISM software. Results: The prevalence of HLA-B61 was significantly higher in idiopathic AA (21.9%, P < 0.0001), hepatitis associated AA (19.2%, P = 0.0089) and fulminant hepatic failure (20%, P = 0.0008) than in healthy Japanese controls (10.7%). No other HLA class I and II alleles including HLA-DR15 significantly differed between children with AA and healthy controls. Furthermore, HLA distribution did not significantly differ between patients who responded completely or partially to immunosuppressive therapy (IST) and non-responders. Conclusions: We did not uncover a significant association between AA and HLA-DR15 in children. Associations between HLA and disease might differ between children and adults with AA. HLA-B61 was positively associated with idiopathic aplastic anemia, hepatitis-associated aplastic anemia and fulminant hepatic failure in pediatric patients. These findings suggest that a common autoimmune mechanism is responsible for both hepatic- and bone marrow-related pathologies in children. Disclosures: No relevant conflicts of interest to declare.

Immunosuppressive therapy using antithymocyte globulin, cyclosporine, and danazol with or without human granulocyte colony-stimulating factor in children with acquired aplastic anemia

Blood ◽  
2000 ◽  
Vol 96 (6) ◽  
pp. 2049-2054 ◽  
Author(s):  
Seiji Kojima ◽  
Shigeyoshi Hibi ◽  
Yoshiyuki Kosaka ◽  
Masuji Yamamoto ◽  
Masahiro Tsuchida ◽  
...  
Keyword(s):  
Aplastic Anemia ◽  
Immunosuppressive Therapy ◽  
Antithymocyte Globulin ◽  
Human Leukocyte ◽  
Initial Response ◽  
Multicenter Trial ◽  
Leukocyte Antigen ◽  
Probability Of Survival ◽  
First Line Therapy ◽  
Febrile Episodes

A prospective multicenter trial of 119 children 1 to 18 years of age with newly diagnosed aplastic anemia (AA) was conducted, comparing treatment using antithymocyte globulin (ATG), cyclosporine (CyA), and danazol (DAN) with or without rhG-CSF (400 μg/m2, day on days 1-90). All children with very severe AA received rhG-CSF (VSAA group, n = 50). The other children were randomized to receive ATG, CyA, DAN, and rhG-CSF (G-CSF+ group, n = 35) or ATG, CyA, and DAN without rhG-CSF (G-CSF− group, n = 34). After 6 months, the hematologic response rate was 71%, 55%, and 77% in the VSAA group, G-CSF+ group, and G-CSF− group, respectively. There was no difference in the incidence of febrile episodes and documented infections between the G-CSF+ and G-CSF− groups. Bone marrow transplantation (BMT) was attempted in 22 patients in whom initial immunosuppressive therapy (IST; n = 18) failed or in whom a relapse occurred after an initial response (n = 4). Nineteen of the 22 patients are alive and well after a median follow-up of 18 months (range, 3 to 66 months) since BMT. The probability of survival at 4 years was 83% ± 7% in the VSAA group, 91% ± 5% in the G-CSF+ group, and 93% ± 6% in the G-CSF− group. Myelodysplastic syndrome (MDS)/acute myeloid leukemia (AML) developed in one patient in each of the three groups; the overall risk for MDS/AML was 3% ± 2% at 4 years. Because the results of IST were encouraging, it is suggested that children with AA receive IST as first-line therapy if there is no human leukocyte antigen-matched sibling donor.

scholarly journals Human leukocyte antigen mismatching and survival after lung transplantation in adult and pediatric patients with cystic fibrosis

2016 ◽  
Vol 151 (2) ◽  
pp. 549-557.e1 ◽  
Author(s):  
Don Hayes ◽  
Jeffery J. Auletta ◽  
Bryan A. Whitson ◽  
Sylvester M. Black ◽  
Stephen Kirkby ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Transplantation ◽  
Human Leukocyte Antigen ◽  
Pediatric Patients ◽  
Human Leukocyte ◽  
Leukocyte Antigen
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