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Immune aplastic anemia (AA) is caused by a type of T cells that destroy hematopoietic stem cells.
Bone marrow failure is successfully treated with hematopoietic cell transplantation or immunosuppressive therapy (IST)
.
Compared with IST alone, EPAG (EPAG) combined with IST produces a higher hematological response and survival rate, but long-term results such as relapse and clonal evolution still have clinical problems, and the biology is still unclear
Immune aplastic anemia (AA) is caused by a type of T cells that destroy hematopoietic stem cells.
Figure 1: Study patients and clinical parameters
Figure 2: Deletion of HLAI class alleles
.
.
Figure 2: Deletion of HLAI class alleles
A research team examined the somatic cell loss of HLA class I alleles and correlated HLA genotypes related to HLA loss and mutation with the clinical manifestations and results of 544 AA patients after immunosuppressive treatment
.
Of the 412 patients tested, 92 (22%) had HLA class I allele loss detected, including 393 somatic HLA gene mutations and 40 instances of loss of heterozygosity
A research team examined the somatic cell loss of HLA class I alleles and correlated HLA genotypes related to HLA loss and mutation with the clinical manifestations and results of 544 AA patients after immunosuppressive treatment
Figure 3: High-risk clone evolution
Figure 4: The clonal evolution of the HLAI class allele 7 lacking clones
.
.
Figure 4: The clonal evolution of the HLAI class allele 7 lacking clones
The most commonly affected is HLA-B*14:02 , followed by HLA-A*02:01 , HLA-B*40:02 , HLA-B*08:01 and HLA-B*07:02
.
The proportions of
The most commonly affected is HLA-B*14:02 , followed by HLA-A*02:01 , HLA-B*40:02 , HLA-B*08:01 and HLA-B*07:02
In two patients, the researchers tracked the evolution of Mono 7 clones that were derived from pre-existing clones that were present in and carried somatic mutations
Overall, this study shows that there are different pathogenic pathways related to HLA genotype and immune AA loss
.
The most important thing in the clinic is the relationship between high-risk malignant clonal evolution and class i-mediated immunity, such as the most commonly inactivated HLA-b*14:02 genotype and general HLA deletion, which provides for this serious complication A simple forecasting tool
.
This study shows that there are different pathogenic pathways related to HLA genotype and immune AA loss
.
The most important thing in the clinic is the relationship between high-risk malignant clonal evolution and class i-mediated immunity, such as the most commonly inactivated HLA-b*14:02 genotype and general HLA deletion, which provides for this serious complication A simple forecasting tool
.
Original source:
Zaimoku Y, Patel BA, Adams SD, Shalhoub RN, Groarke EM, Lee AAC, Kajigaya S, Feng X, Rios OJ, Eager H, Alemu L, Quinones Raffo D, Wu CO, Flegel WA, Young NS.
HLA associations, somatic loss of HLA expression, and clinical outcomes in immune aplastic anemia.
Blood.
2021 Nov 1:blood.
2021012895.
doi: 10.
1182/blood.
2021012895.
Epub ahead of print.
PMID: 34724566.
HLA associations, somatic loss of HLA expression, and clinical outcomes in immune aplastic anemia.
Blood.
2021 Nov 1:blood.
2021012895.
doi: 10.
1182/blood.
2021012895.
Epub ahead of print.
PMID: 34724566.
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