MCID: SPL009
MIFTS: 34

Splenic Sequestration

Categories: Immune diseases

Aliases & Classifications for Splenic Sequestration

MalaCards integrated aliases for Splenic Sequestration:

Name: Splenic Sequestration 12 15 70

Classifications:



External Ids:

Disease Ontology 12 DOID:11786
ICD9CM 34 289.52
UMLS 70 C1260402

Summaries for Splenic Sequestration

MalaCards based summary : Splenic Sequestration is related to sickle cell disease and sickle cell anemia. An important gene associated with Splenic Sequestration is THPO (Thrombopoietin), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and Complement and coagulation cascades. The drugs Hydroxyurea and Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include spleen, bone marrow and liver, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Related Diseases for Splenic Sequestration

Diseases related to Splenic Sequestration via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 237)
# Related Disease Score Top Affiliating Genes
1 sickle cell disease 31.1 VWF HP HBB EPO
2 sickle cell anemia 30.5 VWF ITGA2B HBB F2 EPO
3 hypersplenism 30.4 THPO F2 EPO CD4
4 splenic infarction 30.4 HBB F3 F2
5 hemoglobin c disease 30.4 SCN2A RHCE HBB
6 beta-thalassemia 30.4 HBB F2 EPO CD4
7 hemoglobin e disease 29.9 SCN2A HBB F2
8 exanthem 29.8 F3 F2 CD4
9 hemoglobinopathy 29.7 SCN2A RHCE HP HBB EPO CYGB
10 thrombocytopenia due to platelet alloimmunization 29.6 THPO MPL ITGA2B CD4
11 alpha-thalassemia 29.4 SCN2A ITGA2B HP HBB EPO
12 thrombocytosis 29.2 VWF THPO MPL F3 F2 EPO
13 disseminated intravascular coagulation 29.2 VWF F3 F2 ADAMTS13
14 liver cirrhosis 29.0 THPO HP F3 F2 ADAMTS13
15 thrombocytopenic purpura, autoimmune 28.9 THPO MPL ITGA2B CD4 ADAMTS13
16 portal hypertension 28.7 VWF THPO F3 F2 CD4 ADAMTS13
17 deficiency anemia 28.6 U2AF1 THPO MPL HP HBB F2
18 hemolytic anemia 28.6 VWF RHCE HP HBB F3 F2
19 purpura 28.4 VWF THPO MPL ITGA2B F3 F2
20 thrombocytopenia 28.2 VWF U2AF1 THPO MPL ITGA2B HP
21 sickle cell - hemoglobin d disease 11.2
22 splenomegaly 10.5
23 thalassemia 10.5
24 thrombocytopenia 3 10.4 THPO MPL
25 dyskeratosis congenita, autosomal dominant 6 10.3 THPO MPL
26 acute erythroid leukemia 10.3 HBB EPO
27 intracranial vasospasm 10.3 HP F2
28 thalassemia minor 10.3 HBB EPO
29 anterior spinal artery syndrome 10.3 F2 CYGB
30 refractory anemia 10.2 MPL EPO
31 epiglottis cancer 10.2 EPO CYGB
32 rh isoimmunization 10.2 RHCE EPO
33 sodoku disease 10.2 THPO HP
34 folic acid deficiency anemia 10.2 EPO CYGB
35 achenbach syndrome 10.2 F3 F2
36 thrombocythemia 1 10.2 THPO MPL
37 renal pelvis squamous cell carcinoma 10.2 F3 F2
38 splenic abscess 10.2 F2 CD4
39 synovial angioma 10.2 F3 F2
40 emphysematous cholecystitis 10.2 F3 F2
41 giant hemangioma 10.2 F3 F2
42 hemopneumothorax 10.2 F3 F2
43 dic in newborn 10.2 F3 F2
44 cerebral falx meningioma 10.2 F3 F2
45 ankylosing spondylitis 1 10.2 F3 F2
46 basilar artery occlusion 10.2 ITGA2B F2
47 splenic tuberculosis 10.2 F2 CD4
48 ceftazidime allergy 10.2 EPO CD4
49 waterhouse-friderichsen syndrome 10.2 F3 F2
50 pulmonary embolism and infarction 10.2 F3 F2

Graphical network of the top 20 diseases related to Splenic Sequestration:



Diseases related to Splenic Sequestration

Symptoms & Phenotypes for Splenic Sequestration

GenomeRNAi Phenotypes related to Splenic Sequestration according to GeneCards Suite gene sharing:

26 (show all 23)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-116 9.7 MPL
2 Increased shRNA abundance (Z-score > 2) GR00366-A-120 9.7 U2AF1
3 Increased shRNA abundance (Z-score > 2) GR00366-A-127 9.7 MPL
4 Increased shRNA abundance (Z-score > 2) GR00366-A-13 9.7 ADAMTS13
5 Increased shRNA abundance (Z-score > 2) GR00366-A-136 9.7 U2AF1
6 Increased shRNA abundance (Z-score > 2) GR00366-A-138 9.7 ADAMTS13
7 Increased shRNA abundance (Z-score > 2) GR00366-A-14 9.7 CD4
8 Increased shRNA abundance (Z-score > 2) GR00366-A-141 9.7 MPL
9 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.7 CD4
10 Increased shRNA abundance (Z-score > 2) GR00366-A-153 9.7 U2AF1
11 Increased shRNA abundance (Z-score > 2) GR00366-A-160 9.7 CD4
12 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.7 MPL
13 Increased shRNA abundance (Z-score > 2) GR00366-A-178 9.7 ADAMTS13
14 Increased shRNA abundance (Z-score > 2) GR00366-A-19 9.7 CD4
15 Increased shRNA abundance (Z-score > 2) GR00366-A-211 9.7 U2AF1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.7 MPL
17 Increased shRNA abundance (Z-score > 2) GR00366-A-32 9.7 CD4
18 Increased shRNA abundance (Z-score > 2) GR00366-A-60 9.7 MPL
19 Increased shRNA abundance (Z-score > 2) GR00366-A-73 9.7 MPL
20 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.7 U2AF1
21 Increased shRNA abundance (Z-score > 2) GR00366-A-89 9.7 ADAMTS13 U2AF1
22 Increased shRNA abundance (Z-score > 2) GR00366-A-91 9.7 U2AF1
23 Increased shRNA abundance (Z-score > 2) GR00366-A-97 9.7 ADAMTS13

MGI Mouse Phenotypes related to Splenic Sequestration:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.85 ADAMTS13 CD4 EPO F3 HP ITGA2B
2 homeostasis/metabolism MP:0005376 9.73 ADAMTS13 CD4 CYGB EPO F2 F3
3 immune system MP:0005387 9.32 ADAMTS13 CD4 EPO F2 F3 HP

Drugs & Therapeutics for Splenic Sequestration

Drugs for Splenic Sequestration (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Hydroxyurea Approved Phase 2 127-07-1 3657
2 Immunoglobulins Phase 2
3 Antibodies, Monoclonal Phase 2
4 Antibodies Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase II, Multicenter, Randomized, Placebo-Controlled, Double-Blind, 12-Month Study to Assess Safety and Efficacy of SelG1 With or Without Hydroxyurea Therapy in Sickle Cell Disease Patients With Sickle Cell-Related Pain Crises Completed NCT01895361 Phase 2 SelG1;Placebo
2 Study of Prognostic Factors of Acute Splenic Sequestration in a Cohort of Sickle Cell Disease (SCD) Children Diagnosed at Birth Completed NCT01207037

Search NIH Clinical Center for Splenic Sequestration

Genetic Tests for Splenic Sequestration

Anatomical Context for Splenic Sequestration

MalaCards organs/tissues related to Splenic Sequestration:

40
Spleen, Bone Marrow, Liver, Myeloid

Publications for Splenic Sequestration

Articles related to Splenic Sequestration:

(show top 50) (show all 445)
# Title Authors PMID Year
1
The spectrum of splenic complications in patients with sickle cell disease in Africa: a systematic review. 61
33161568 2021
2
Red cell transfusion and alloimmunization in sickle cell disease. 61
33792218 2021
3
Impact of the coronavirus 2019 (COVID-19) on vasooclusive crisis in patients with sickle cell anaemia. 61
33741487 2021
4
Transjugular intrahepatic portosystemic shunt creation may be associated with hyperplastic hepatic nodular lesions in the long term: an analysis of 18 pediatric and young adult patients. 61
33783576 2021
5
Case Report of Acute Splenic Sequestration Crisis in an Adult Patient with Hb S Disease and Suspected Hereditary Persistence of Fetal Hemoglobin. 61
33588663 2021
6
Splenic sequestration in the adult: cross sectional imaging appearance of an uncommon diagnosis. 61
33070084 2021
7
Platelet phenotype and function in the absence of splenic sequestration (Review). 61
32106750 2021
8
Massive Splenomegaly and Pancytopenia: It's a Hairy Situation. 61
33500860 2020
9
Failure of CD4 T Cell-Deficient Hosts To Control Chronic Nontyphoidal Salmonella Infection Leads to Exacerbated Inflammation, Chronic Anemia, and Altered Myelopoiesis. 61
33046510 2020
10
A pediatric patient with sickle cell disease presenting with severe anemia and splenic sequestration in the setting of COVID-19. 61
32776396 2020
11
Acute Splenic Sequestration Crisis in Hemoglobin SC Disease: Efficiency of Red Cell Exchange. 61
33500853 2020
12
Optical coherence tomography (OCT) and OCT angiography allow early identification of sickle cell maculopathy in children and correlate it with systemic risk factors. 61
32518974 2020
13
Autologous platelet scintigraphy and clinical outcome of splenectomy in immune thrombocytopenia: A systematic review and meta-analysis. 61
32712518 2020
14
[Sickle cell trait complications: A case series of 6 patients]. 61
32768266 2020
15
The risk of acute events among patients with sickle cell disease in relation to early or late initiation of care at a specialist center: evidence from a retrospective cohort study. 61
32770992 2020
16
Thrombocytopenia in Chronic Liver Disease: New Management Strategies. 61
32620282 2020
17
Prevalence and predictive factors of splenic sequestration crisis among 423 pediatric patients with sickle cell disease in Tunisia. 61
31670184 2020
18
Hb S/β-Thalassemia in the REDS-III Brazil Sickle Cell Disease Cohort: Clinical, Laboratory and Molecular Characteristics. 61
32172616 2020
19
[Diagnosis of anemia associated with alcoholic cirrhosis]. 61
31609556 2019
20
Open Versus Laparoscopic Splenectomies in Children: A Comparative Study Performed at a Public Hospital in Brazil. 61
31539304 2019
21
Prospective longitudinal follow-up of children with sickle cell disease treated with hydroxyurea since infancy. 61
31157521 2019
22
Insights into determinants of spleen injury in sickle cell anemia. 61
31391165 2019
23
The clinical characteristics of Congolese children and adolescents suffering from sickle-cell anemia are marked by the high frequencies of epistaxis compared to Western series. 61
31339399 2019
24
Considering the spleen in sickle cell disease. 61
31195851 2019
25
Co-inheritance of a-thalassemia dramatically decreases the risk of acute splenic sequestration in a large cohort of newborns with hemoglobin SC. 61
30630972 2019
26
Splenic sequestration crisis as an index manifestation of heterozygous hemoglobinopathy in an adult. 61
31312463 2019
27
Sickle cell disease: Reducing the global disease burden. 61
31069977 2019
28
The Sub-Phenotypes of Sickle Cell Disease in Kuwait. 61
31144996 2019
29
Trends in Hospitalizations for Sickle Cell Disease Related-Complications in USA 2004 - 2012. 61
32300435 2019
30
Gastro-splenic fistula, a rare presentation of sickle cell disease patient with splenic abscess: Case report and literature review. 61
30875625 2019
31
Sickle cell diseases: What can nuclear medicine offer? 61
30843001 2019
32
Evaluation of Vaso-occlusive Crises in United States Sickle Cell Disease Patients: A Retrospective Claims-based Study. 61
32685584 2019
33
A Differential Approach to an Uncommon Case of Acute Anemia in a Child With Sickle Cell Disease. 61
31106251 2019
34
Clinical and hematological profile in a newborn cohort with hemoglobin SC. 61
29195085 2018
35
Prognostic factors of disease severity in infants with sickle cell anemia: A comprehensive longitudinal cohort study. 61
30132969 2018
36
Refractoriness to red blood cell transfusion therapy due to hypersplenism. 61
30260482 2018
37
Sensing of red blood cells with decreased membrane deformability by the human spleen. 61
30305267 2018
38
The Addition of Bevacizumab to Oxaliplatin-Based Chemotherapy: Impact Upon Hepatic Sinusoidal Injury and Thrombocytopenia. 61
29346573 2018
39
Clinical and Imaging Predictors of Surgical Splenorenal Shunt Dysfunction in Pediatric Patients. 61
29470285 2018
40
Fatal Sickling Triggered by Massive Foreign Particle Embolism: A Case Report of Unrecognized Indwelling Venous Catheter Drug Abuse in Sickle Cell Disease. 61
29351102 2018
41
Acute Splenic Sequestration Crisis After Red Blood Cell Exchange for Acute Chest Syndrome in an Adult With Sickle β-Thalassemia: What Went Wrong? 61
29205801 2018
42
Ceftriaxone-induced drug reaction mimicking acute splenic sequestration crisis in a child with hemoglobin SC disease. 61
29473172 2018
43
Folate supplementation in people with sickle cell disease. 61
29546732 2018
44
An analysis of inpatient pediatric sickle cell disease: Incidence, costs, and outcomes. 61
28801954 2018
45
Causes of death and early life determinants of survival in homozygous sickle cell disease: The Jamaican cohort study from birth. 61
29494636 2018
46
Association of the SOD2 Polymorphism (Val16Ala) and SOD Activity with Vaso-occlusive Crisis and Acute Splenic Sequestration in Children with Sickle Cell Anemia. 61
29531649 2018
47
Recent advances in understanding and management of acquired thrombocytopenia. 61
29399327 2018
48
Myelofibrosis-Related Anemia: Current and Emerging Therapeutic Strategies. 61
31723730 2017
49
Novel use Of Hydroxyurea in an African Region with Malaria (NOHARM): a trial for children with sickle cell anemia. 61
29051184 2017
50
Biofunctionalized Hybrid Magnetic Gold Nanoparticles as Catalysts for Photothermal Ablation of Colorectal Liver Metastases. 61
28707960 2017

Variations for Splenic Sequestration

Expression for Splenic Sequestration

Search GEO for disease gene expression data for Splenic Sequestration.

Pathways for Splenic Sequestration

GO Terms for Splenic Sequestration

Cellular components related to Splenic Sequestration according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.92 VWF THPO HP HBB F3 F2
2 extracellular space GO:0005615 9.76 VWF THPO HP HBB F3 F2
3 endocytic vesicle lumen GO:0071682 9.37 HP HBB
4 haptoglobin-hemoglobin complex GO:0031838 9.26 HP HBB
5 blood microparticle GO:0072562 9.26 ITGA2B HP HBB F2
6 cell surface GO:0009986 9.1 MPL ITGA2B F3 EPO CD4 ADAMTS13

Biological processes related to Splenic Sequestration according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 platelet activation GO:0030168 9.58 VWF F2 ADAMTS13
2 cellular oxidant detoxification GO:0098869 9.54 HP HBB CYGB
3 blood coagulation, intrinsic pathway GO:0007597 9.46 VWF F2
4 oxygen transport GO:0015671 9.43 HBB CYGB
5 platelet aggregation GO:0070527 9.43 MPL ITGA2B HBB
6 blood coagulation GO:0007596 9.35 VWF HBB F3 F2 ADAMTS13
7 acute-phase response GO:0006953 9.33 HP F2 EPO
8 thrombopoietin-mediated signaling pathway GO:0038163 9.32 THPO MPL
9 hemostasis GO:0007599 8.92 VWF F3 F2 ADAMTS13

Molecular functions related to Splenic Sequestration according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 oxygen carrier activity GO:0005344 9.16 HBB CYGB
2 hemoglobin binding GO:0030492 8.96 HP HBB
3 immunoglobulin binding GO:0019865 8.62 VWF CD4

Sources for Splenic Sequestration

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
Content
Loading form....