PMID- 16263420
OWN - NLM
STAT- MEDLINE
DCOM- 20060112
LR  - 20071115
IS  - 0301-472X (Print)
IS  - 0301-472X (Linking)
VI  - 33
IP  - 11
DP  - 2005 Nov
TI  - Killer immunoglobulin-like receptor genotype in immune-mediated bone marrow 
      failure syndromes.
PG  - 1357-62
AB  - OBJECTIVE: Recent reports have shown that killer immunoglobulin-like receptors 
      (KIR) and KIR ligand (KIR-L) genotype play a role in the pathophysiology of 
      autoimmune disorders. Certain KIR/KIR-L combinations might convey a 
      predisposition to immune-mediated bone marrow failure. Examining the genotypic 
      makeup of human leukocyte antigen (HLA) class I (KIR-L) and KIR in patients with 
      hematologic disorders could offer clues to immunogenetic risk factors for these 
      diseases. The objective of this study is to establish the frequency of specific 
      KIR genes and KIR-L alleles in aplastic anemia (AA), paroxysmal nocturnal 
      hemoglobinuria (PNH), myelodysplasia (MDS), and large granular lymphocyte 
      leukemia (LGL), as compared with healthy control patients. METHODS: KIR 
      genotyping was performed on DNA from 113 patients with AA, PNH, MDS, and LGL 
      using sequence specific primer amplification. Results of genotypic KIR analysis 
      were examined with HLA typing. Comparisons in frequency of KIR-L groups, KIR 
      genotype profiles, and KIR/KIR-L mismatch were performed to determine whether 
      specific KIR genes and KIR-L are overrepresented in bone marrow failure states. 
      RESULTS: No difference in frequency of KIR-L groups was found relative to the 
      healthy controls. AA and PNH showed decreased frequency of KIR-2DS1 and KIR-2DS5 
      genes: KIR-2DS1 35% vs 21% (p = 0.15) for AA and 19% for PNH (p = 0.13); KIR-2DS5 
      31% vs 14% (p = 0.08) for AA and 12% (p = 0.06) for PNH. These differences were 
      even greater when compared to a larger group of control individuals from a study 
      with similar genetic background. CONCLUSIONS: The reduced frequency of these KIR 
      in AA and PNH may indicate an immunogenetic relationship between these diseases.
FAU - Howe, Evan C
AU  - Howe EC
AD  - Experimental Hematology and Hematopoiesis Section, Taussig Cancer Center, 
      Cleveland, OH 44195, USA.
FAU - Wlodarski, Marcin
AU  - Wlodarski M
FAU - Ball, Edward J
AU  - Ball EJ
FAU - Rybicki, Lisa
AU  - Rybicki L
FAU - Maciejewski, Jaroslaw P
AU  - Maciejewski JP
LA  - eng
PT  - Journal Article
PL  - Netherlands
TA  - Exp Hematol
JT  - Experimental hematology
JID - 0402313
RN  - 0 (Histocompatibility Antigens Class I)
RN  - 0 (Ligands)
RN  - 0 (Receptors, Immunologic)
RN  - 0 (Receptors, KIR)
SB  - IM
MH  - Anemia, Aplastic/genetics/immunology
MH  - Bone Marrow Diseases/genetics/*immunology
MH  - Case-Control Studies
MH  - Gene Frequency
MH  - Genotype
MH  - Hemoglobinuria, Paroxysmal/genetics/immunology
MH  - Histocompatibility Antigens Class I/genetics
MH  - Humans
MH  - Immunity
MH  - Leukemia, Lymphoid/genetics/immunology
MH  - Ligands
MH  - Myelodysplastic Syndromes/genetics/immunology
MH  - Receptors, Immunologic/*genetics
MH  - Receptors, KIR
MH  - Syndrome
EDAT- 2005/11/03 09:00
MHDA- 2006/01/13 09:00
CRDT- 2005/11/03 09:00
PHST- 2005/04/13 00:00 [received]
PHST- 2005/06/06 00:00 [revised]
PHST- 2005/07/01 00:00 [accepted]
PHST- 2005/11/03 09:00 [pubmed]
PHST- 2006/01/13 09:00 [medline]
PHST- 2005/11/03 09:00 [entrez]
AID - S0301-472X(05)00362-0 [pii]
AID - 10.1016/j.exphem.2005.07.005 [doi]
PST - ppublish
SO  - Exp Hematol. 2005 Nov;33(11):1357-62. doi: 10.1016/j.exphem.2005.07.005.