MCID: SPL018
MIFTS: 47

Splenomegaly malady

Rare diseases category
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Summaries for Splenomegaly

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Wikipedia:65 Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ)... more...

MalaCards based summary: Splenomegaly is related to hypersplenism and myelofibrosis. An important gene associated with Splenomegaly is CHIT1 (chitinase 1 (chitotriosidase)), and among its related pathways are Lysosome and Jak/Stat Pathway. The compounds miglustat and megapoietin have been mentioned in the context of this disorder. Affiliated tissues include spleen, bone and bone marrow, and related mouse phenotypes are cardiovascular system and liver/biliary system.

Aliases & Classifications for Splenomegaly

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Sources:
42NIH Rare Diseases, 44Novoseek, 25ICD10
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Related Diseases for Splenomegaly

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Diseases related to Splenomegaly via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 362)
idRelated DiseaseScoreTop Affiliating Genes
1hypersplenism31.2GBA, THPO
2myelofibrosis30.9THPO, MPL
3gaucher's disease30.8CHIT1, GBA
4polycythemia vera30.6THPO, MPL
5thrombocythemia 130.5THPO
6polycythemia30.5MPL, THPO
7purpura30.5THPO
8cholesteryl ester storage disease30.3LIPA
9neutropenia30.3THPO, MPL
10pancytopenia30.3HP, MPL, THPO, GBA
11niemann-pick disease30.2LCAT, CHIT1
12refractory anemia30.2THPO, MPL
13hemolytic anemia30.2LCAT, HP
14leukemia30.1HP, MPL, THPO, GBA
15thrombocytosis30.1HP, MPL, THPO
16fabry disease30.1CHIT1, GBA
17hepatitis c30.1HP, THPO
18thrombocytopenia30.1GBA, THPO, MPL, CHIT1, HP
19myelodysplastic syndromes29.9MPL, THPO
20liver cirrhosis29.6THPO, MPL, HP, LCAT
21deficiency anemia28.6GBA, THPO, MPL, CHIT1, HP, LCAT
22chitotriosidase deficiency10.5CHIT1
23hepatitis10.4
24sarcoidosis10.4
25hypertension10.4
26malaria10.4
27portal hypertension10.3
28felty's syndrome10.3
29banti's syndrome10.3
30myeloid splenomegaly10.3
31gaucher disease type 110.3CHIT1, GBA
32hypoalphalipoproteinemia10.3LCAT
33pyridoxine-refractory autosomal recessive sideroblastic anemia10.2GBA, MPL
34plasmodium falciparum malaria10.2CHIT1, HP
35congenital amegakaryocytic thrombocytopenia10.2THPO, MPL
36thrombocytopenia due to platelet alloimmunization10.2MPL, THPO
37mantle cell lymphoma10.2
38chronic congestive splenomegaly10.2
39visceral leishmaniasis10.2
40leishmaniasis10.2
41pancreatitis10.2
42autoimmune thrombocytopenic purpura10.2MPL, THPO
43hemorrhagic thrombocythemia10.2MPL, THPO
44hepatitis e10.2
45infectious mononucleosis10.2
46schistosomiasis10.2
47aplastic anemia10.1MPL, THPO
48hepatoblastoma10.1THPO, MPL
49bone marrow cancer10.1THPO, MPL
50arthritis10.1

Graphical network of the top 20 diseases related to Splenomegaly:



Diseases related to splenomegaly

Symptoms for Splenomegaly

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Drugs & Therapeutics for Splenomegaly

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Drug clinical trials:

Search ClinicalTrials for Splenomegaly

Search NIH Clinical Center for Splenomegaly

Genetic Tests for Splenomegaly

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Anatomical Context for Splenomegaly

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MalaCards organs/tissues related to Splenomegaly:

32
Spleen, Bone, Bone marrow, Liver, Myeloid, B cells, T cells, Colon, Skin, Testes, Lung, Endothelial, Eye, Lymph node, Small intestine, Kidney, Thyroid, Uterus, Prostate, Cervix, Monocytes, Tongue

Animal Models for Splenomegaly or affiliated genes

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MGI Mouse Phenotypes related to Splenomegaly:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.2LCAT, HP, LIPA, GBA
2MP:00053708.1GBA, LIPA, HP, LCAT
3MP:00053877.6GBA, THPO, MPL, LIPA, CHIT1, HP
4MP:00053767.4GBA, MPL, LIPA, HP, LCAT
5MP:00053977.2LCAT, HP, LIPA, MPL, THPO, GBA

Publications for Splenomegaly

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Articles related to Splenomegaly:

(show top 50)    (show all 578)
idTitleAuthorsYear
1
Assessment of immune cells and function of the residual spleen after subtotal splenectomy due to splenomegaly in cirrhotic patients. (25293512)
2014
2
Stunted growth, splenomegaly, and interstitial pneumopathy. (25173787)
2014
3
Is platelet transfusion necessary in cirrhotic patients with splenomegaly? (24102873)
2014
4
Bartonella henselae infection in a man with hypergammaglobulinaemia, splenomegaly and polyclonal plasmacytosis. (23118473)
2013
5
Isolated splenomegaly as the only presentation of systemic mastocytosis. (23604430)
2013
6
A case of liver fibrosis with splenomegaly after oxaliplatin-based adjuvant chemotherapy for colon cancer. (24339718)
2013
7
Gigantic splenomegaly in a 27-year-old male of South-East Asian descent with concurrent diagnosis of myeloproliferative neoplasm and hemoglobin H disease. (23672282)
2013
8
Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: a case of extramedullary hematopoiesis. (21922646)
2012
9
Synchronous presentation of acute pancreatitis and splenomegaly with intussusceptions in Peutz-Jeghers syndrome. (22925293)
2012
10
Prophylaxis against hepatitis B virus recurrence after liver transplantation for hepatitis B virus-related end-stage liver diseases with severe hypersplenism and splenomegaly: role of splenectomy. (22483806)
2012
11
Small cell variant of mantle cell lymphoma is an indolent lymphoma characterized by bone marrow involvement, splenomegaly, and a low Ki-67 index. (21609366)
2011
12
Seventy-four-year-old woman with myelodysplastic syndrome and splenomegaly. (20615327)
2010
13
A febrile young infant with splenomegaly and ecchymoses. (20531132)
2010
14
Splenectomy in two children with autoimmune lymphoproliferative syndrome and massive splenomegaly. (19499578)
2009
15
Detection of splenomegaly on Meckel's diverticulum scan. (19352291)
2009
16
An unexpected cause of a febrile patient with huge splenomegaly. (18365137)
2008
17
Radiation therapy for palliation of Eisenmenger's syndrome-associated painful splenomegaly. (18301984)
2008
18
Progressive splenomegaly following splenic artery embolization. (18481504)
2008
19
Case records of the Massachusetts General Hospital. Case 39-2008. A 51-year-old woman with splenomegaly and anemia. (19092156)
2008
20
Splenomegaly and Sports. (22908407)
2008
21
Haematological parameters in sickle cell anaemia patients with and without splenomegaly. (16380738)
2005
22
Splenomegaly, anaemia and pleural effusion. (15632233)
2005
23
Determination of splenomegaly by CT: is there a place for a single measurement? (15855107)
2005
24
Conjugation of 3' hexameric deoxyriboguanosine run to phosphodiester CpG oligodeoxynucleotides can inhibit allergen-specific IgE synthesis with less risk of splenomegaly. (16337478)
2005
25
Elevated plasma levels of vascular endothelial growth factor is associated with marked splenomegaly in chronic myeloid leukemia. (16263579)
2005
26
Sarcoidosis manifesting as massive splenomegaly: a rare occurrence. (15367876)
2004
27
Hand-assisted laparoscopic splenectomy in the setting of splenomegaly. (15803233)
2004
28
Congenital absence of the portal vein with splenomegaly and hypersplenism in a young woman. (12743449)
2003
29
Combined needlescopic cholecystectomy and laparoscopic splenectomy for the treatment of thalassaemic splenomegaly and cholelithiasis. (14750388)
2003
30
Acute hypersplenism with splenomegaly after portal vein embolization. (12075192)
2002
31
Laparoscopic splenectomy for massive splenomegaly. (11918887)
2002
32
Hand-assisted laparoscopic splenectomy vs conventional laparoscopic splenectomy in cases of splenomegaly. (12470097)
2002
33
Littoral cell angioma as a rare cause of splenomegaly. (11233776)
2001
34
Splenomegaly, hypersplenism and coagulation abnormalities in liver disease. (11139352)
2000
35
Mantle cell lymphoma, in leukaemic phase with prominent splenomegaly. A report of eight cases with similar clinical presentation and aggressive outcome. (11193469)
2000
36
Laparoscopically assisted splenectomy following preoperative splenic artery embolization using contour emboli for myelofibrosis with massive splenomegaly. (10804000)
1999
37
Fever, thrombocytopenia and splenomegaly in a neonate. (9951993)
1999
38
Splenomegaly, eosinophilia, and pruritus: Hodgkin's disease, or...? (9269801)
1997
39
Comparative study between different diagnostic techniques in huge splenomegaly cases in El-Minia Province. (17214200)
1995
40
Splenomegaly in Baringo District, Kenya, an area endemic for visceral leishmaniasis and malaria. (7483000)
1995
41
Splenomegaly and percussion: is the background noise due to tongue in cheek? (8046799)
1994
42
Persistence of portosystemic collaterals and splenomegaly on CT after orthotopic liver transplantation. (1632346)
1992
43
Rapid aggravation of splenomegaly by administration of erythropoietin in a case of myelodysplastic syndrome]. (1479699)
1992
44
Pharmacologic manipulation of graft versus host induced splenomegaly. (1529801)
1992
45
Vesicular rash, radicular pain, and splenomegaly in a patient with Lyme borreliosis. (1319756)
1992
46
Systemic cystic angiomatosis in a woman with hematuria and splenomegaly. (3607642)
1987
47
Control of hypersplenism and painful splenomegaly in myeloid metaplasia by irradiation. (413815)
1977
48
Pycnodysostosis with splenomegaly and anemia. (5286691)
1971
49
SPIKING ACID HYPERPHOSPHATASIA, SPLENOMEGALY, AND LEUKEMOID REACTION DURING A HEMOLYTIC SICKLE CELL CRISIS. (14167678)
1964
50
The hemorrhagic tendency in congestive splenomegaly (Banti's syndrome); its mechanism and management. (18899129)
1948

Variations for Splenomegaly

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Expression for genes affiliated with Splenomegaly

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Expression patterns in normal tissues for genes affiliated with Splenomegaly

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Pathways for genes affiliated with Splenomegaly

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Pathways related to Splenomegaly according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5LIPA, GBA
2
Show member pathways
Development Thrombopoetin signaling via JAK STAT pathway60
9.4MPL, THPO
3
Show member pathways
9.4MPL, THPO

Compounds for genes affiliated with Splenomegaly

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Sources:
44Novoseek, 11DrugBank, 24HMDB, 50PharmGKB, 28IUPHAR
See all sources

Compounds related to Splenomegaly according to GeneCards/GeneDecks:

(show top 50)    (show all 71)
idCompoundScoreTop Affiliating Genes
1miglustat44 1111.1GBA, CHIT1
2megapoietin449.9MPL, THPO
3Cholesteryl acetate249.6LCAT, LIPA
4anagrelide44 1110.6MPL, THPO
5CE(20:4(8Z,11Z,14Z,17Z))249.6LIPA, LCAT
6CE(18:3(9Z,12Z,15Z))249.5LCAT, LIPA
7CE(20:5(5Z,8Z,11Z,14Z,17Z)249.5LCAT, LIPA
8CE(19:0)249.5LCAT, LIPA
9CE(18:3(6Z,9Z,12Z))249.5LIPA, LCAT
10CE(20:1(11Z))249.5LCAT, LIPA
11CE(20:4(5Z,8Z,11Z,14Z)249.5LIPA, LCAT
12CE(20:2(6Z,9Z))249.5LCAT, LIPA
13CE(20:3(5Z,8Z,11Z))249.5LCAT, LIPA
14CE(20:0)249.5LIPA, LCAT
15CE(20:3(8Z,11Z,14Z))249.5LCAT, LIPA
16CE(22:0)249.5LCAT, LIPA
17CE(22:2(13Z,16Z))249.5LCAT, LIPA
18CE(24:0)249.5LIPA, LCAT
19CE(22:6(4Z,7Z,10Z,13Z,16Z,19Z))249.5LIPA, LCAT
20CE(22:4(7Z,10Z,13Z,16Z)249.5LCAT, LIPA
21CE(22:5(4Z,7Z,10Z,13Z,16Z))249.5LIPA, LCAT
22CE(22:1(13Z))249.5LIPA, LCAT
23CE(22:5(7Z,10Z,13Z,16Z,19Z))249.5LCAT, LIPA
24CE(5:0)249.5LIPA, LCAT
25CE(24:1(15Z))249.4LIPA, LCAT
26CE(18:2(9Z,12Z))249.4LIPA, LCAT
27CE(14:1(9Z))249.4LIPA, LCAT
28CE(14:0)249.4LIPA, LCAT
29CE(12:0)249.4LIPA, LCAT
30CE(10:0)249.4LIPA, LCAT
31CE(16:0)249.4LCAT, LIPA
32polyethylene glycol449.4THPO, HP, MPL
33CE(18:1(9Z))249.4LIPA, LCAT
34gp 130449.3MPL, THPO, HP
35CE(18:1(11Z))249.3LIPA, LCAT
36CE(18:0)249.3LIPA, LCAT
37CE(16:1(9Z))249.3LIPA, LCAT
38cholesterol ester449.1LCAT, LIPA, HP
39mannose449.0GBA, LIPA, CHIT1
40iron44 2410.0HP, MPL, THPO, CHIT1
41polysaccharide448.9THPO, GBA, HP, CHIT1
42aspirin44 50 28 2411.9HP, THPO, GBA, MPL
43adenylate448.8LIPA, GBA, HP, THPO
44phospholipid448.6LCAT, GBA, HP, LIPA
45lipid448.2GBA, LCAT, HP, CHIT1, LIPA
46cholesterol44 28 24 1111.2LIPA, HP, GBA, LCAT
47alanine448.1LCAT, HP, THPO, MPL
48steroid448.1LIPA, THPO, GBA, HP
49serine447.8HP, LIPA, MPL, GBA, LCAT
50cysteine447.6LCAT, GBA, THPO, MPL, LIPA, HP

GO Terms for genes affiliated with Splenomegaly

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Cellular components related to Splenomegaly according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056158.1LCAT, HP, CHIT1, THPO

Biological processes related to Splenomegaly according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cell proliferationGO:0082838.9LIPA, MPL, THPO

Products for genes affiliated with Splenomegaly

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  • Antibodies
  • Proteins
  • Lysates

Sources for Splenomegaly

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet