PMID- 35491217
OWN - NLM
STAT- MEDLINE
DCOM- 20220503
LR  - 20220503
IS  - 0485-1439 (Print)
IS  - 0485-1439 (Linking)
VI  - 63
IP  - 4
DP  - 2022
TI  - [Immune-mediated thrombotic thrombocytopenic purpura and susceptible HLA 
      alleles].
PG  - 277-285
LID - 10.11406/rinketsu.63.277 [doi]
AB  - Thrombotic thrombocytopenic purpura (TTP) is an extremely rare and fatal 
      thrombotic disorder characterized by impaired enzyme activity of von Willebrand 
      factor cleaving protease, also known as ADAMTS13. Immune-mediated TTP (iTTP) is 
      an acquired form of TTP caused by the production of auto-antibodies against 
      ADAMTS13. The pathophysiology of autoimmune disorders is multifactorial, with 
      several human leukocyte antigen (HLA) alleles identified as a genetic risk factor 
      for autoimmune diseases known as susceptible HLA. In the early 2010s, three 
      distinct European groups revealed that DRB1(*)11 is one of the most susceptible 
      alleles in acquiring iTTP among Caucasians based on HLA typing data. Several in 
      silico predictions for allele-restricted ADAMTS13 epitopes against T cells are 
      made in this context, followed by an in vitro validation employing mass 
      spectrometry using eluted peptides and T-cell assays. However, similar analyses 
      in a genetically distinct Japanese population have not yet been conducted. We 
      used next-generation sequencing to perform HLA typing for 52 Japanese patients 
      with iTTP from 19 institutes. Our detailed analysis revealed that the specific 
      allele DRB1(*)08:03 was identified as a genetic risk factor for iTTP in Japanese 
      patients, but there were no statistically significant differences in the allele 
      frequency of DRB1(*)11 between iTTP and healthy controls.
FAU - Sakai, Kazuya
AU  - Sakai K
AD  - Department of Blood Transfusion Medicine, Nara Medical University.
LA  - jpn
PT  - Journal Article
PL  - Japan
TA  - Rinsho Ketsueki
JT  - [Rinsho ketsueki] The Japanese journal of clinical hematology
JID - 2984782R
SB  - IM
MH  - Alleles
MH  - Disease Susceptibility/complications
MH  - Histocompatibility Testing/adverse effects
MH  - Humans
MH  - *Purpura, Thrombotic Thrombocytopenic/genetics
MH  - Risk Factors
OTO - NOTNLM
OT  - ADAMTS13
OT  - Allele-restricted T-cell epitopes
OT  - Immune-mediated thrombotic thrombocytopenic purpura
OT  - Susceptible HLA
EDAT- 2022/05/02 06:00
MHDA- 2022/05/04 06:00
CRDT- 2022/05/01 21:54
PHST- 2022/05/01 21:54 [entrez]
PHST- 2022/05/02 06:00 [pubmed]
PHST- 2022/05/04 06:00 [medline]
AID - 10.11406/rinketsu.63.277 [doi]
PST - ppublish
SO  - Rinsho Ketsueki. 2022;63(4):277-285. doi: 10.11406/rinketsu.63.277.