Table 2.

Association of KIR genotypes and haematological malignancies

KIR genotype and associations with haematological malignancies
KIR gene	Disease	Effect	No patients in study	References
Risk of disease
KIR-2DS1 KIR-2DS5	Aplastic anaemia / Paroxysmal nocturnal haemoglobinuria	Reduced frequency in patient population	55	(125)
KIR2DS3	AML/ALL	Reduced frequency in patient population	40/32	(126)
Activating KIR genes, especially KIR2DS2	Pre-B ALL	Reduced frequency in patient population	102	(127)
KIR3DS1 and KIR2DS1	Hodgkin’s Lymphoma	Reduced frequency in patients	84 families	(128)
KIR2DL2 and KIR2DS2	CML	Reduced frequency in patients	52	(129)
KIR2DL2 and KIR2DL3 in combination with their HLA-Cw1 ligand	CML/AML	Increased frequency in patients	19/29	(31)
KIR2DL5a and 2DL5b	NK type lymphoma	Increased frequency in patients	35	(26)
KIR3DL1 and the HLA-Bw4-ligand	CLL	Increased frequency in patients in the presence of Bw4 and reduced risk when Bw4 absent.	31	(31)
Response to treatment
KIR2DS1	CML	Reduced response to imatinib and worse overall survival. Dasatinib therapy overcomes the poor prognostic impact of KIR2DS1.	166 + validated a further 174 on SPIRIT2	(34) (35)
KIR3DS1	Myeloma	Worse DFS following autologous stem cell transplant.	182	(28)
Donor KIR B/B homozygotes	AML	Reduced risk of relapse following unrelated allogeneic transplant (relapse rate 15.4% B/B v 36.5% A/A)	1086	(130)
Donor KIR 2DS1 (plus coinherited 3DS1 and 2DL5a)	AML	Reduced relapse rates following HLA-identical sibling allogeneic stem cell transplant (relapse rate 13% with SD1+ve donor v 57% if −ve)	248 patients: 68 with AML	(33)
Donor KIR 2DS1	AML	Reduced relapse rates following HLA-identical unrelated donor allogeneic stem cell transplant (relapse rate 26.5% with 2DS1+ donor v 32.5% 2DS1−ve	1277	(32)
Donor KIR haplotype B	Myeloma	Improved progression free survival (30 v 14%)	118	(67)