Paroxysmal Nocturnal Hemoglobinuria malady

NIH Rare Diseases: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder that leads to the premature death and impaired production of blood cells. It can occur at any age, but is usually diagnosed in young adulthood. People with PNH have recurring episodes of symptoms due to hemolysis, which may be triggered by stresses on the body such as infections or physical exertion. This results in a deficiency of various types of blood cells and can cause signs and symptoms such as fatigue, weakness, abnormally pale skin (pallor), shortness of breath, and an increased heart rate. People with PNH may also be prone to infections and abnormal blood clotting (thrombosis) or hemorrhage, and are at increased risk of developing leukemia. It is caused by acquired, rather than inherited, mutations in the PIGA gene; the condition is not passed down to children of affected individuals. Sometimes, people who have been treated for aplastic anemia may develop PNH. The treatment of PNH is largely based on symptoms; stem cell transplantation is typically reserved for severe cases of PNH with aplastic anemia or those whose develop leukemia.³⁰

MalaCards: Paroxysmal Nocturnal Hemoglobinuria, also known as hemoglobinuria, paroxysmal, is related to aplastic anemia and hematopoiesis. An important gene associated with Paroxysmal Nocturnal Hemoglobinuria is PIGA (phosphatidylinositol glycan anchor biosynthesis, class A), and among its related pathways are Immune response_Lectin induced complement pathway and Glycosylphosphatidylinositol(GPI)-anchor biosynthesis. The compounds rituximab and hirudin have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and bone marrow, and related mouse phenotypes are liver/biliary system and respiratory system.

Genetics Home Reference: Paroxysmal nocturnal hemoglobinuria is an acquired disorder that leads to the premature death and impaired production of blood cells. The disorder affects red blood cells (erythrocytes), which carry oxygen; white blood cells (leukocytes), which protect the body from infection; and platelets (thrombocytes), which are involved in blood clotting. Paroxysmal nocturnal hemoglobinuria affects both sexes equally, and can occur at any age, although it is most often diagnosed in young adulthood.¹⁷

Wikipedia: Paroxysmal nocturnal hemoglobinuria (PNH), sometimes referred to as Marchiafava-Micheli syndrome, is a...⁴⁴ more...

paroxysmal nocturnal hemoglobinuria 7 30 17 43 hemoglobinuria, paroxysmal 17 43 paroxysmal hemoglobinuria nocturnal 32 marchiafava-micheli syndrome 17

marchiafava-micheli disease 30 hemoglobinuria 43 pnh 30

Diseases related to paroxysmal nocturnal hemoglobinuria by text searches and GeneDecks gene sharing:

(show top 50)    (show all 445)

id	Related Disease	Score	Top Affiliating Genes
1	aplastic anemia	35.5	PIGA, CD58, CD59, CD55, HPRT1, TFRC
2	hematopoiesis	32.9	PIGA, CD14, HPRT1, HMGA2, WT1, TFRC
3	pancytopenia	32.5	PIGA, CD59, CD55, HP, WT1, CSF3
4	myelodysplastic syndrome	32.2	PIGA, CD59, CD55, ANXA5, HPRT1, NRAS
5	myelofibrosis	31.1	CEACAM8, HMGA2, PLAUR, WT1, CSF3, EPO
6	retinal vascular occlusion	30.6	PIGA, SELP, MTHFR
7	sepsis	30.0	RECK, SELP, CD14, CD55, FCGR3B, F10
8	protein s deficiency	30.0	PIGA, RECK, MTHFR, CD58, FCGR3B, PLCG1
9	venous thrombosis	28.9	VWF, SELP, MTHFR, F10, HLA-B
10	duodenitis	27.9	CD14, CD59, CD55, HLA-A, HLA-B, HLA-DQA1
11	hemoglobinuria	27.7	PIGA, PIGF, PIGH, RECK, KIR2DS4, VWF
12	anemia	27.2	PIGA, VWF, SELP, MTHFR, CD58, CD14
13	thrombosis	26.8	PIGA, RECK, VWF, SELP, MTHFR, CD14
14	was-related disorders	26.2	PIGA, HLA-B, WT1, ACHE, PRNP, CSF3
15	acute leukemia	24.7	VWF, SELP, MTHFR, CD58, CD14, CD59
16	retinitis	23.7	PIGA, PIGF, VWF, SELP, MTHFR, ANXA5
17	acute myocardial infarction	23.4	VWF, SELP, MTHFR, CD14, ANXA5, F10
18	myocardial infarction	22.6	RECK, VWF, SELP, MTHFR, CD14, CD59
19	leukemia	21.0	PIGA, RECK, KIR2DS4, VWF, SELP, LBR
20	x-linked sideroblastic anemia	13.7	PIGA, CD59, CD55
21	t-lymphocytopenia	13.6	LBR, CD59, ANXA5
22	dyserythropoietic anemia with thrombocytopenia	13.4	PIGA, CD59, CD55, CSF3, EPO
23	hemoglobinopathy	13.4	HP, TFRC, EPO
24	birdshot chorioretinopathy	13.3	KIR2DS4, HLA-A, HLA-C
25	hairy cell leukemia	13.2	RECK, CD52, ANXA5, CSF3, EPO
26	quebec platelet disorder	13.2	SELP, F10, PLAU
27	beta thalassemia	13.2	ANXA5, F10, TFRC, EPO
28	chronic lymphocytic leukemia	13.2	CD58, CD59, CD52, CD55, ANXA5, TFRC
29	autoimmune thyroiditis	13.1	CD58, CD59, CD55, HLA-B
30	t cell deficiency	13.1	PIGA, ANXA5, HPRT1, PLCG1, PLAUR
31	autoimmune hemolytic anemia	13.1	PIGA, VWF, CD59, CD52, CD55, HP
32	blood platelet disease	13.1	RECK, VWF, F10
33	infective endocarditis	13.1	VWF, CEACAM8, EPO
34	pyelonephritis	13.1	CD55, HLA-A, CSF3, EPO
35	afibrinogenemia	13.1	VWF, F10, C3
36	lymphoproliferative syndrome	13.0	LBR, CD59, CD52, CD55, HPRT1, HLA-A
37	glanzmann's thrombasthenia	13.0	RECK, VWF, F10
38	extrinsic allergic alveolitis	13.0	CD14, HP, HLA-DRB1
39	seminoma	13.0	PLAU, PLAUR, GFRA1, CSF3, EPO
40	iga glomerulonephritis	13.0	LBR, CD14, CD55, F10, TFRC, C3
41	oral lichen planus	13.0	CD58, CD14, FCGR3B, HLA-A, HLA-C
42	pelvic inflammatory disease	13.0	HLA-DQA1, HLA-DQB1, PLAU
43	al amyloidosis	13.0	VWF, CD14, F10, CSF3
44	hemochromatosis	13.0	HP, HLA-B, TFRC
45	sympathetic ophthalmia	12.9	HLA-C, HLA-DQA1, HLA-DRB1
46	psoriatic arthritis	12.9	CD58, HLA-C, HLA-DRB1
47	iron deficiency anemia	12.9	SELP, TFRC, CSF3, EPO
48	head injury	12.9	SELP, HP, ACHE, EPO
49	thrombotic thrombocytopenic purpura, acquired	12.8	VWF, SELP, HP
50	pyruvate kinase deficiency	12.8	MTHFR, ANXA5, EPO

Approved drugs:

Drug clinical trials:

MalaCards organs/tissues related to paroxysmal nocturnal hemoglobinuria:

²²

MGI Mouse Phenotypes related to paroxysmal nocturnal hemoglobinuria:

²⁵ (show all 14)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	liver/biliary system phenotype	MP:0005370	9.7	C3, RECK, HPRT1, PLG, EPO
2	respiratory system phenotype	MP:0005388	9.3	ACHE, HPRT1, SELP, CD14
3	renal/urinary system phenotype	MP:0005367	8.8	PLG, HPRT1, C3, CD55, SELP, GFRA1
4	hematopoietic system phenotype	MP:0005397	8.6	NRAS, HLA-DQB1, HLA-C, KLRK1, TFRC, CSF3
5	integument phenotype	MP:0010771	8.6	SELP, PIGA, CSF3, GFRA1, PLCG1, RECK
6	reproductive system phenotype	MP:0005389	8.2	PLG, HMGA2, PRNP, HPRT1, F10, CD59
7	digestive/alimentary phenotype	MP:0005381	8.2	TFRC, C3, GFRA1, PLG, PLAU, NRAS
8	growth/size phenotype	MP:0005378	7.0	SELP, LBR, MTHFR, CD55, HPRT1, PLCG1
9	cardiovascular system phenotype	MP:0005385	6.8	TFRC, WT1, PLG, PLAUR, PLAU, PLCG1
10	immune system phenotype	MP:0005387	6.8	PLG, PLAUR, WT1, GFRA1, TFRC, PRNP
11	nervous system phenotype	MP:0003631	6.5	VWF, C3, SELP, PIGA, RECK, PLG
12	cellular phenotype	MP:0005384	6.0	HPRT1, C3, HLA-DQB1, NRAS, PLCG1, PLAU
13	homeostasis/metabolism phenotype	MP:0005376	5.6	MTHFR, LBR, SELP, VWF, HP, PLAU
14	mortality/aging	MP:0010768	4.7	PIGA, KLRK1, EPO, NRAS, HLA-C, HPRT1

Articles related to paroxysmal nocturnal hemoglobinuria:

(show top 50)    (show all 188)

id	Title	Authors	Year	Affiliating Genes
1	Enhanced expression of CD71, transferrin receptor, on immature reticulocytes in patients with paroxysmal nocturnal hemoglobinuria. (19302232)	Sato S.... Ninomiya H.	2010	TFRC
2	Eculizumab prevents intravascular hemolysis in patien ts with paroxysmal nocturnal hemoglobinuria and unmasks low-level extravascular hemolysis occurring through C3 opsonization. (20145265)	Hill A.... Hillmen P.	2010	HP
3	Paroxysmal nocturnal hemoglobinuria (19303177)	Peffault de Latour R.... SociAc G.	2010	CD59, PIGA
4	Detection of paroxysmal nocturnal hemoglobinuria clones in patients with myelodysplastic syndromes and related bone marrow diseases, with emphasis on diagnostic pitfalls and caveats. (19001281)	Wang S.A.... Woda B.A.	2009	CD55, CD59
5	Paroxysmal nocturnal hemoglobinuria may cause retinal vascular occlusions. (18437293)	Scheuerle A.F.... Beutelspacher S.C.	2009	PIGA
6	Paroxysmal nocturnal hemoglobinuria. (19080854)	HernA!ndez-Campo P.M.... Orfao A.	2008	PIGA
7	Haptoglobin therapy during pregnancy for paroxysmal nocturnal hemoglobinuria with renal failure. (17459392)	Shibasaki T.... Furuya K.	2007	HP
8	Erythopoietin treatment during complement inhibition with eculizumab in a patient with paroxysmal nocturnal hemoglobinuria. (17405753)	Hill A.... Hillmen P.	2007	EPO
9	Molecular basis of clonal expansion of hematopoiesis in 2 patients with paroxysmal nocturnal hemoglobinuria (PNH). (16940417)	Inoue N.... Kinoshita T.	2006	PIGA, HMGA2
10	PIG-A mutations in paroxysmal nocturnal hemoglobinuria and in normal hematopoiesis. (16923549)	Brodsky R.A.... Hu R.	2006	PIGA
11	Ultrastructural study of platelets patients with paroxysmal nocturnal hemoglobinuria (17408109)	Vega S.... Cerecedo-Mercado D.A.	2006	SELP
12	CD55 and CD59 deficiency in transplant patient populations: possible association with paroxysmal nocturnal hemoglobinuria-like symptoms in Campath-treated patients. (16908271)	Ruiz P.... Tzakis A.	2006	CD55, CD59
13	A cohort study of the nature of paroxysmal nocturnal hemoglobinuria clones and PIG-A mutations in patients with aplastic anemia. (16529603)	Wanachiwanawin W.... Kinoshita T.	2006	PIGA
14	Budd-Chiari syndrome in a paroxysmal nocturnal hemoglobinuria patient with coexistence of factor II and MTHFR mutations. (15869626)	Shamseddine A.I.... Taher A.T.	2005	MTHFR
15	Diazepam-binding inhibitor-related protein 1: a candidate autoantigen in acquired aplastic anemia patients harboring a minor population of paroxysmal nocturnal hemoglobinuria-type cells. (15217832)	Feng X.... Nakao S.	2004	ECI2
16	Increased resistance of PIG-A- bone marrow progenitors to tumor necrosis factor a and interferon gamma: possible implications for the in vivo dominance of paroxysmal nocturnal hemoglobinuria clones. (15194531)	Barcellini W.... Zanella A.	2004	PIGA
17	Expression of CD59 on lymphocyte and the subsets and its potential clinical application for paroxysmal nocturnal hemoglobinuria diagnosis. (15053802)	Cui W.... Zhang Z.	2004	CD59
18	Laboratory diagnosis of paroxysmal nocturnal hemoglobinuria. (14584753)	Krauss J.S.	2003	PIGA
19	Recent insights into the pathophysiology of paroxysmal nocturnal hemoglobinuria. (12883466)	Meletis J.... Terpos E.	2003	PIGA
20	A SIN lentiviral vector containing PIGA cDNA allows long-term phenotypic correction of CD34+-derived cells from patients with paroxysmal nocturnal hemoglobinuria. (12668126)	Robert D.... Moreau-Gaudry F.	2003	PIGA
21	Phenotypes and phosphatidylinositol glycan-class A gene abnormalities during cell differentiation and maturation from precursor cells to mature granulocytes in patients with paroxysmal nocturnal hemoglobinuria. (12411324)	Kai T.... Maruyama Y.	2002	CD59, PIGA
22	History of paroxysmal nocturnal hemoglobinuria (19569576)	ChrobA!k L.	2002	PIGA
23	Activated platelets of patients with paroxysmal nocturnal hemoglobinuria express cellular prion protein. (12070046)	Holada K.... Vostal J.G.	2002	PRNP
24	Red cells with paroxysmal nocturnal hemoglobinuria-phenotype in patients with acute leukemia. (12186694)	Meletis J.... Konstantopoulos K.	2002	CD55, CD59
25	Successful unrelated donor bone marrow transplantatio n for paroxysmal nocturnal hemoglobinuria. (11319587)	Woodard P.... Bowman L.	2001	PIGA
26	Detection of somatic mutations of the PIG-A gene in Brazilian patients with paroxysmal nocturnal hemoglobinuria. (11378665)	Franco De Carvalho R.... Costa F.F.	2001	PIGA
27	N-RAS gene mutation in patients with aplastic anemia and aplastic anemia/ paroxysmal nocturnal hemoglobinuria during evolution to clonal disease. (10627475)	Mortazavi Y.... Rutherford T.R.	2000	NRAS, PIGA
28	Paroxysmal nocturnal hemoglobinuria (membrane defect, pathogenesis, aplastic anemia, diagnosis). (10934778)	ChrobA!k L.	2000	PIGA
29	The molecular basis of paroxysmal nocturnal hemoglobinuria. (10629597)	Rosti V.	2000	PIGA
30	Resistance of paroxysmal nocturnal hemoglobinuria cells to the glycosylphosphatidylinositol-binding toxin aerolysin. (10029605)	Brodsky R.A.... Buckley J.T.	1999	PIGA
31	Clonal populations of hematopoietic cells with paroxysmal nocturnal hemoglobinuria genotype and phenotype are present in normal individuals. (10220445)	Araten D.J.... Luzzatto L.	1999	PIGA
32	Paroxysmal nocturnal hemoglobinuria and its association with aplastic anemia (9748736)	Pavlu J.... Necas E.	1998	PIGA
33	Paroxysmal nocturnal hemoglobinuria as a molecular disease. (9100736)	Rosse W.F.	1997	PIGA
34	Paroxysmal nocturnal hemoglobinuria: efficacy of prolonged treatment with granulocyte colony-stimulating factor. (9326254)	JAcgo P.... DrAcnou B.	1997	CSF3
35	Analysis of PIG-A gene in a patient who developed reciprocal translocation of chromosome 12 and paroxysmal nocturnal hemoglobinuria during follow-up of aplastic anemia. (8619404)	Nishimura J.I.... Takeda J.	1996	PIGA
36	Role of phosphatidylinositol-linked proteins in paroxysmal nocturnal hemoglobinuria pathogenesis. (8712763)	Kinoshita T.... Takeda J.	1996	PIGA
37	Paroxysmal nocturnal hemoglobinuria: the price for a chance. (8946596)	Bessler M.	1996	PIGA
38	Molecular basis of the heterogeneity of expression of glycosyl phosphatidylinositol anchored proteins in paroxysmal nocturnal hemoglobinuria. (8630422)	Endo M.... Parker C.J.	1996	PIGA
39	Paroxysmal nocturnal hemoglobinuria clone in bone marrow of patients with pancytopenia. (7532041)	Nakakuma H.... Takatsuki K.	1995	CD55, CD59
40	The PIG-A gene somatic mutation responsible for paroxysmal nocturnal hemoglobinuria. (8647522)	Rotoli B.... Boccuni P.	1995	PIGA
41	Negative Ham's test and sugar water test on admissio n in paroxysmal nocturnal hemoglobinuria (7967061)	Noji H.... Maruyama Y.	1994	HP
42	Chromosomal assignment of genes involved in glycosylphosphatidylinositol anchor biosynthesis: implications for the pathogenesis of paroxysmal nocturnal hemoglobinuria. (8204896)	Ware R.E.... Seldin M.F.	1994	PIGF, PIGH
43	Characterization of alternatively spliced PIG-A transcripts in normal and paroxysmal nocturnal hemoglobinuria cells. (8081230)	Yu J.... Medof M.E.	1994	PIGA
44	Expression of recombinant transmembrane CD59 in paroxysmal nocturnal hemoglobinuria B cells confers resistance to human complement. (7522635)	Rother R.P.... Squinto S.P.	1994	CD55, CD59
45	Recent advances in research on paroxysmal nocturnal hemoglobinuria (8315823)	Kinoshita T.	1993	PIGA
46	Expression of decay-accelerating factor and CD59 in lymphocyte subsets of healthy individuals and paroxysmal nocturnal hemoglobinuria patients. (7686331)	Nagakura S.... Takatsuki K.	1993	CD55, CD59
47	Complement-induced vesiculation and exposure of membrane prothrombinase sites in platelets of paroxysmal nocturnal hemoglobinuria. (7688991)	Wiedmer T.... Sims P.J.	1993	F10
48	Assembly and deacetylation of N-acetylglucosaminyl-plasmanylinositol in normal and affected paroxysmal nocturnal hemoglobinuria cells. (1708886)	Hirose S.... Medof M.E.	1991	PLCG1, GPLD1
49	Synthesis of aberrant decay-accelerating factor proteins by affected paroxysmal nocturnal hemoglobinuria leukocytes. (1688570)	Carothers D.J.... Medof M.E.	1990	CD55
50	Analysis of PI (phosphatidylinositol)-anchoring antigens in a patient of paroxysmal nocturnal hemoglobinuria (PNH) reveals deficiency of 1F5 antigen (CD59), a new complement-regulatory factor. (1689670)	Taguchi R.... Nakashima I.	1990	CD59

Genes related to paroxysmal nocturnal hemoglobinuria according to GeneCards:

(show all 47)

id	Symbol	Description	Score	GeneCards Section Context
1	PIGA	phosphatidylinositol glycan anchor biosynthesis, class A	11.1	Publications, Aliases & Descriptions, Orthologs, Summaries
2	CD59	CD59 molecule, complement regulatory protein	11.0	Publications
3	CEACAM8	carcinoembryonic antigen-related cell adhesion molecule 8	11.0	Publications
4	PIGH	phosphatidylinositol glycan anchor biosynthesis, class H	11.0	Publications
5	ECI2	enoyl-CoA delta isomerase 2	11.0	Publications
6	KLRC2	killer cell lectin-like receptor subfamily C, member 2	11.0	Publications
7	CD52	CD52 molecule	11.0	Publications
8	FCGR3B	Fc fragment of IgG, low affinity IIIb, receptor (CD16b)	11.0	Publications
9	PIGF	phosphatidylinositol glycan anchor biosynthesis, class F	11.0	Publications
10	CD55	CD55 molecule, decay accelerating factor for complement (Cromer blood group)	11.0	Publications
11	SEMA7A	semaphorin 7A, GPI membrane anchor (John Milton Hagen blood group)	11.0	Publications
12	ALDH7A1	aldehyde dehydrogenase 7 family, member A1	11.0	Publications
13	KIR2DS4	killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 4	11.0	Publications
14	HP	haptoglobin	11.0	Publications
15	GPLD1	glycosylphosphatidylinositol specific phospholipase D1	11.0	Publications
16	KLRK1	killer cell lectin-like receptor subfamily K, member 1	11.0	Publications
17	CD58	CD58 molecule	11.0	Publications
18	NRAS	neuroblastoma RAS viral (v-ras) oncogene homolog	11.0	Publications
19	C9	complement component 9	11.0	Publications
20	PLAUR	plasminogen activator, urokinase receptor	11.0	Publications
21	PLAU	plasminogen activator, urokinase	11.0	Publications
22	RECK	reversion-inducing-cysteine-rich protein with kazal motifs	11.0	Publications
23	LBR	lamin B receptor	11.0	Publications
24	HMGA2	high mobility group AT-hook 2	11.0	Publications
25	C3	complement component 3	11.0	Publications
26	CD14	CD14 molecule	11.0	Publications
27	CSF3	colony stimulating factor 3 (granulocyte)	11.0	Publications
28	PLCG1	phospholipase C, gamma 1	11.0	Publications
29	EPO	erythropoietin	11.0	Publications
30	PLG	plasminogen	11.0	Publications
31	WT1	Wilms tumor 1	11.0	Publications
32	TFRC	transferrin receptor (p90, CD71)	11.0	Publications
33	ANXA5	annexin A5	11.0	Publications
34	PRNP	prion protein	11.0	Publications
35	F10	coagulation factor X	11.0	Publications
36	HPRT1	hypoxanthine phosphoribosyltransferase 1	11.0	Publications
37	HLA-DQA1	major histocompatibility complex, class II, DQ alpha 1	11.0	Publications
38	HLA-C	major histocompatibility complex, class I, C	11.0	Publications
39	GFRA1	GDNF family receptor alpha 1	11.0	Publications
40	ACHE	acetylcholinesterase	11.0	Publications
41	HLA-DQB1	major histocompatibility complex, class II, DQ beta 1	11.0	Publications
42	SELP	selectin P (granule membrane protein 140kDa, antigen CD62)	11.0	Publications
43	HLA-A	major histocompatibility complex, class I, A	11.0	Publications
44	MTHFR	methylenetetrahydrofolate reductase (NAD(P)H)	11.0	Publications
45	HLA-B	major histocompatibility complex, class I, B	11.0	Publications
46	HLA-DRB1	major histocompatibility complex, class II, DR beta 1	11.0	Publications
47	VWF	von Willebrand factor	11.0	Publications

Pathways related to paroxysmal nocturnal hemoglobinuria according to GeneDecks:

(show all 39)

id	Pathway	Score	Top Affiliating Genes
1	Immune response_Lectin induced complement pathway41	10.5	CD59, CD55, C9
2	Glycosylphosphatidylinositol(GPI)-anchor biosynthesis20	10.4	GPLD1, PIGA, PIGF, PIGH
3	Immune response_Alternative complement pathway41	10.4	C3, CD55, C9, CD59
4	Immune response Alternative complement pathway10	10.3	C3, C9, CD59, CD55
5	Cell adhesion molecules (CAMs)20	10.3	CD58, SELP
6	Immune response Lectin Induced complement pathway10	10.3	CD59, C3, C9, CD55
7	Immune response Classic complement pathway10	10.3	C3, C9, CD55, CD59
8	Immune response_Classical complement pathway41	10.2	C3, C9, CD55, CD59
9	Blood Coagulation Signaling Pathways37	9.7	VWF, PLG, PLAU, F10
10	Immune response_Role of DAP12 receptors in NK cells41	9.6	KLRC2, HLA-B, HLA-C
11	Cell surface interactions at the vascular wall38	9.5	NRAS, PLCG1, SELP, CD58
12	Immune response Role of DAP12 receptors in NK cells10	9.5	HLA-C, PLCG1, KLRC2, HLA-B
13	Graft-versus-host disease20	9.2	HLA-A, HLA-B, HLA-C, HLA-DQA1
14	Complement and coagulation cascades20	9.1	C9, F10, PLAU, CD59, PLG, PLAUR
15	IL-4 Pathway36	9.1	HLA-DQA1, HLA-DQB1, HLA-DRB1, NRAS
16	Hematopoietic cell lineage20	9.1	CD14, CD59, CD55, HLA-DRB1, TFRC, CSF3
17	Calcium Mediated T-Cell Apoptosis36	9.1	HLA-DRB1, HLA-DQB1, HLA-DQA1, PLCG1
18	Leishmaniasis20	9.1	HLA-DQB1, C3, HLA-DRB1, FCGR3B, HLA-DQA1
19	MHC class II antigen presentation38	9.1	HLA-DQA1, HLA-DQB1, HLA-DRB1
20	G-protein signaling N-RAS regulation pathway10	9.1	HLA-DQA1, HLA-DRB1, NRAS, PLCG1, HLA-DQB1
21	G-protein signaling_N-RAS regulation pathway41	9.0	HLA-DQA1, PLCG1, NRAS, HLA-DRB1, HLA-DQB1
22	Systemic lupus erythematosus20	8.9	C3, C9, HLA-DQB1, HLA-DRB1, HLA-DQA1, FCGR3B
23	CTLA4 Signaling36	8.9	HLA-DQA1, HLA-DQB1, PLCG1, HLA-DRB1
24	PKC-Theta Pathway36	8.9	HLA-DQB1, NRAS, HLA-DRB1, HLA-DQA1, PLCG1
25	Antigen processing and presentation20	8.9	HLA-DQA1, KLRC2, HLA-DRB1, HLA-DQB1, HLA-C, KIR2DS4
26	Tuberculosis20	8.9	HLA-DRB1, CD14, FCGR3B, HLA-DQA1, HLA-DQB1, C3
27	TCR Signaling36	8.8	HLA-DQA1, HLA-DRB1, HLA-DQB1, NRAS, PLCG1
28	Immune response T cell receptor signaling pathway10	8.8	PLCG1, HLA-DQA1, HLA-DQB1, HLA-DRB1
29	Immune response_T cell receptor signaling pathway41	8.8	HLA-DRB1, PLCG1, HLA-DQA1, HLA-DQB1
30	Natural killer cell mediated cytotoxicity20	8.8	KLRK1, KLRC2, PLCG1, NRAS, HLA-C, HLA-B
31	Staphylococcus aureus infection20	8.5	FCGR3B, HLA-DQA1, HLA-DQB1, SELP, HLA-DRB1, PLG
32	ICos-ICosL Pathway in T-Helper Cell36	8.5	PLCG1, HLA-DRB1, HLA-DQB1, HLA-DQA1
33	CTL Mediated Apoptosis36	8.0	HLA-DQB1, HLA-A, HLA-DQA1, HLA-C, HLA-B, HLA-DRB1
34	Allograft rejection20	8.0	HLA-DRB1, HLA-B, HLA-A, HLA-DQB1, HLA-DQA1, HLA-C
35	Type I diabetes mellitus20	8.0	HLA-DQB1, HLA-DRB1, HLA-DQA1, HLA-C, HLA-B, HLA-A
36	Autoimmune thyroid disease20	8.0	HLA-A, HLA-DQA1, HLA-DQB1, HLA-DRB1, HLA-B, HLA-C
37	Viral myocarditis20	7.9	HLA-C, CD55, HLA-A, HLA-B, HLA-DQB1, HLA-DRB1
38	CDC42 Pathway36	7.9	HLA-C, HLA-DQB1, HLA-DQA1, HLA-B, HLA-A, HLA-DRB1
39	Phagosome20	7.4	CD14, C3, FCGR3B, HLA-A, HLA-B, HLA-C

Compounds related to paroxysmal nocturnal hemoglobinuria according to GeneDecks:

(show top 50)    (show all 157)

id	Compound	Score	Top Affiliating Genes
1	rituximab32 34 9 9	13.5	CD52, CD59, FCGR3B, CD55
2	hirudin32	10.3	F10, PLAU, PLAUR
3	dmso32	9.9	PRNP, HPRT1, SELP
4	sialic acid32	9.9	RECK, EPO, TFRC, HP, GPLD1, CD55
5	methylcellulose32	9.7	EPO, CSF3, PIGA, CD59, ANXA5, CD14
6	neopterin32	9.7	LBR, HP, PLAUR, TFRC, C3, CD14
7	pge232	9.7	PRNP, PLAUR, PLCG1, HP, CD14, PLAU
8	fluorochrome32	9.6	SELP, ANXA5, PLAUR, TFRC, CD14
9	tranexamic acid32 9 9	11.6	EPO, PLG, PLAUR, VWF, PLAU
10	phosphatidylserine32 9 9	11.6	FCGR3B, RECK, VWF, CD14, F10
11	abciximab32 9 9	11.5	FCGR3B, VWF, ANXA5, F10
12	fmlp32	9.4	CD14, CSF3, PLCG1, FCGR3B, CD55, CD59
13	ascorbic acid32 18	10.3	EPO, TFRC, F10, ANXA5, HPRT1
14	glycolipid32	9.2	RECK, CD14, CD59, CD55, PRNP, HLA-B
15	kininogen32	9.2	VWF, HP, PLAUR, PLG, PLAU, SELP
16	glutamate32	9.2	PLAU, ANXA5, PLCG1, MTHFR, HPRT1, F10
17	aprotinin32 9 9	11.0	F10, SELP, VWF, PLCG1, PLAUR, PLAU
18	aspartate32	9.0	SELP, CD14, HLA-DQA1, F10, HP, PLAU
19	fitc32	8.9	TFRC, HLA-B, ANXA5, CD55, VWF, CD59
20	mycophenolate mofetil32 9 9	10.9	CSF3, TFRC, HPRT1, SELP, EPO
21	agarose32	8.8	C3, VWF, PRNP, HP, GPLD1, ANXA5
22	vitamin d32	8.8	CD14, LBR, EPO, TFRC, WT1, PLAUR
23	phospholipid32	8.8	PLCG1, GPLD1, C3, HP, F10, ANXA5
24	polyacrylamide32	8.7	HLA-DQA1, RECK, CD14, GPLD1, HP, HLA-B
25	folate32	8.6	TFRC, EPO, VWF, RECK, PLCG1, HPRT1
26	heparin32 9 18 9	11.6	PRNP, PLG, PLCG1, RECK, VWF, LBR
27	carbohydrates32	8.6	HLA-B, PLAUR, TFRC, RECK, SELP, F10
28	aspirin32 34 18	10.6	CD59, MTHFR, SELP, VWF, HP, PLAU
29	h2o232	8.5	F10, GPLD1, VWF, HP, HPRT1, PLCG1
30	histamine32 18	9.4	SELP, PLCG1, CD55, F10, CD14, LBR
31	thymidine32 18	9.3	TFRC, WT1, PLG, PLAUR, PLAU, CSF3
32	phosphatidylinositol32	8.3	CD59, ANXA5, FCGR3B, CD55, CD14, CD58
33	fibrinogen32	8.3	RECK, SELP, CD14, ANXA5, F10, HP
34	glutamine32	8.2	PLAUR, WT1, TFRC, VWF, PLAU, PLCG1
35	cisplatin32 34 9 9	11.2	PLAU, HMGA2, CSF3, EPO, TFRC, PLAUR
36	cysteine32	8.1	C3, GFRA1, PLAUR, PLCG1, CD55, CD14
37	paraffin32	8.1	CD14, HLA-DQA1, TFRC, PRNP, WT1, PLAUR
38	polysaccharide32	7.8	RECK, SELP, CD14, CD52, CD55, FCGR3B
39	lipid32	7.6	C3, PRNP, CD59, GPLD1, ANXA5, FCGR3B
40	lactate32	7.6	CD59, SELP, F10, RECK, CD14, ANXA5
41	vegf32	7.4	ANXA5, CD55, CD14, SELP, VWF, RECK
42	cholesterol32 9 18 9	10.3	RECK, VWF, SELP, LBR, MTHFR, CD14
43	dexamethasone32 42 34 9 9	11.3	ANXA5, CD55, HP, HLA-B, PLCG1, PLAUR
44	creatinine32	7.1	EPO, C3, TFRC, PLAUR, PLAU, WT1
45	alanine32	6.9	CD55, PLCG1, HLA-DRB1, HLA-A, PLAUR, TFRC
46	retinoic acid32 42 18	8.6	KIR2DS4, VWF, SELP, LBR, MTHFR, CD58
47	cyclosporin a32 42	7.4	VWF, SELP, LBR, MTHFR, RECK, CD14
48	serine32	6.0	PLG, PRNP, HP, ACHE, WT1, PLAUR
49	arginine32	5.5	HPRT1, F10, CD14, LBR, SELP, RECK
50	tyrosine32	4.3	PIGF, NRAS, PLCG1, PLAU, PLAUR, PLG

Cellular components related to paroxysmal nocturnal hemoglobinuria according to GeneDecks:

(show all 12)

id	Name	GO ID	Score	Top Affiliating Genes
1	Golgi membrane	GO:000139	9.8	HLA-DQB1, HLA-DQA1
2	MHC class I protein complex	GO:042612	9.1	HLA-C, HLA-B, HLA-A
3	MHC class II protein complex	GO:042613	9.1	HLA-DRB1, HLA-DQB1, HLA-DQA1
4	extracellular space	GO:005615	8.8	C3, HP, PLG, GPLD1, CEACAM8, SELP
5	anchored to membrane	GO:031225	8.8	CD52, GFRA1, RECK, SEMA7A, CEACAM8, CD58
6	clathrin-coated endocytic vesicle membrane	GO:030669	8.7	HLA-DRB1, HLA-DQB1, HLA-DQA1
7	external side of plasma membrane	GO:009897	8.3	HLA-DRB1, ANXA5, SEMA7A, VWF, SELP
8	integral to lumenal side of endoplasmic reticulum membrane	GO:071556	7.8	HLA-C, HLA-B, HLA-A, HLA-DQB1, HLA-DRB1, HLA-DQA1
9	ER to Golgi transport vesicle membrane	GO:012507	7.8	HLA-A, HLA-DRB1, HLA-DQB1, HLA-DQA1, HLA-C, HLA-B
10	extracellular region	GO:005576	7.4	HP, TFRC, CD59, CD14, VWF, C9
11	integral to plasma membrane	GO:005887	6.7	HLA-C, HLA-B, CD52, HLA-DQA1, HLA-DRB1, PLAUR
12	plasma membrane	GO:005886	4.2	CD59, CD14, CD58, CEACAM8, SEMA7A, SELP

Biological processes related to paroxysmal nocturnal hemoglobinuria according to GeneDecks:

(show all 11)

id	Name	GO ID	Score	Top Affiliating Genes
1	C-terminal protein lipidation	GO:006501	10.2	PIGH, PIGF, PLAUR, PIGA
2	preassembly of GPI anchor in ER membrane	GO:016254	10.2	PIGH, PIGF, PIGA
3	GPI anchor biosynthetic process	GO:006506	9.9	PIGA, PIGH, PIGF
4	fibrinolysis	GO:042730	9.9	PLAU, PLG, PLAUR
5	antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independent	GO:002480	9.2	HLA-A, HLA-C, HLA-B
6	T cell receptor signaling pathway	GO:050852	8.8	HLA-DQA1, PLCG1, HLA-DQB1, HLA-DRB1
7	detection of bacterium	GO:016045	8.6	HLA-A, HLA-B, HLA-DRB1
8	blood coagulation	GO:007596	8.1	CD58, SELP, C9, CD59, ANXA5, F10
9	interferon-gamma-mediated signaling pathway	GO:060333	7.8	HLA-DRB1, HLA-DQB1, HLA-A, HLA-B, HLA-C, HLA-DQA1
10	cytokine-mediated signaling pathway	GO:019221	7.4	HLA-B, HLA-C, HLA-DQA1, HLA-DRB1, CSF3, HLA-DQB1
11	immune response	GO:006955	7.2	HLA-B, FCGR3B, HLA-A, HLA-C, HLA-DQA1, HLA-DQB1

Molecular functions related to paroxysmal nocturnal hemoglobinuria according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	eukaryotic cell surface binding	GO:043499	9.3	EPO, SELP, ANXA5
2	MHC class II receptor activity	GO:032395	9.0	HLA-DRB1, HLA-DQB1, HLA-DQA1
3	MHC class I receptor activity	GO:032393	9.0	HLA-B, HLA-A, HLA-C
4	protein binding	GO:005515	5.8	EPO, C3, PRNP, ACHE, KLRC2, WT1