MCID: HPT070
MIFTS: 42

Hepatosplenic T-Cell Lymphoma

Categories: Blood diseases, Cancer diseases, Immune diseases, Liver diseases, Rare diseases

Aliases & Classifications for Hepatosplenic T-Cell Lymphoma

MalaCards integrated aliases for Hepatosplenic T-Cell Lymphoma:

Name: Hepatosplenic T-Cell Lymphoma 59 73

Characteristics:

Orphanet epidemiological data:

59
hepatosplenic t-cell lymphoma
Age of onset: Adolescent,Adult;

Classifications:



External Ids:

Orphanet 59 ORPHA86882
UMLS via Orphanet 74 C1333984
ICD10 via Orphanet 34 C86.1
ICD10 33 C86.1
UMLS 73 C1333984

Summaries for Hepatosplenic T-Cell Lymphoma

MalaCards based summary : Hepatosplenic T-Cell Lymphoma is related to lymphoma and crohn's disease. An important gene associated with Hepatosplenic T-Cell Lymphoma is ABCB1 (ATP Binding Cassette Subfamily B Member 1). Affiliated tissues include spleen, t cells and bone.

Wikipedia : 76 Hepatosplenic T-cell lymphoma is a rare and generally incurable form of lymphoma, except in the case of... more...

Related Diseases for Hepatosplenic T-Cell Lymphoma

Diseases related to Hepatosplenic T-Cell Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 56)
# Related Disease Score Top Affiliating Genes
1 lymphoma 11.1
2 crohn's disease 10.5
3 leukemia 10.5
4 inflammatory bowel disease 10.4
5 lymphocytic leukemia 10.3
6 acute leukemia 10.3
7 hemolytic anemia 10.2
8 hepatitis 10.2
9 thrombocytopenia 10.2
10 helsmoortel-van der aa syndrome 10.1 ABCB1 CD7
11 spondyloarthropathy 1 10.0
12 systemic lupus erythematosus 10.0
13 myelofibrosis 10.0
14 leukemia, acute myeloid 10.0
15 myelodysplastic syndrome 10.0
16 autoimmune lymphoproliferative syndrome, type v 10.0
17 autoimmune hepatitis 10.0
18 common variable immunodeficiency 10.0
19 glomerulonephritis 10.0
20 hematopoietic stem cell transplantation 10.0
21 hemophagocytic lymphohistiocytosis 10.0
22 hepatitis b 10.0
23 schistosomiasis 10.0
24 membranoproliferative glomerulonephritis 10.0
25 purpura 10.0
26 progressive multifocal leukoencephalopathy 10.0
27 spondylitis 10.0
28 t-cell leukemia 10.0
29 myeloid leukemia 10.0
30 lupus erythematosus 10.0
31 ring chromosome 7 10.0
32 splenomegaly 10.0
33 orbit lymphoma 10.0 GZMB TIA1
34 lichen sclerosus et atrophicus 10.0 GZMB TIA1
35 lymphomatoid granulomatosis 10.0 GZMB TIA1
36 viral esophagitis 10.0 GZMB TIA1
37 lymphocytic gastritis 10.0 GZMB TIA1
38 subcutaneous panniculitis-like t-cell lymphoma 10.0 GZMB TIA1
39 panniculitis 10.0 GZMB TIA1
40 primary cutaneous anaplastic large cell lymphoma 10.0 GZMB TIA1
41 composite lymphoma 10.0 GZMB TIA1
42 lymphomatoid papulosis 10.0 GZMB TIA1
43 natural killer cell leukemia 10.0 GZMB TIA1
44 choriocarcinoma of the testis 10.0 GZMB TIA1
45 sezary's disease 9.9 CD7 DPP4
46 giardiasis 9.9 GZMB TIA1
47 infiltrating lipoma 9.9 GZMB TIA1
48 necrotizing sialometaplasia 9.9 GZMB TIA1
49 lymphoma, non-hodgkin, familial 9.9 ABCB1 CD7 GZMB
50 ileum cancer 9.9 GZMB TIA1

Graphical network of the top 20 diseases related to Hepatosplenic T-Cell Lymphoma:



Diseases related to Hepatosplenic T-Cell Lymphoma

Symptoms & Phenotypes for Hepatosplenic T-Cell Lymphoma

Drugs & Therapeutics for Hepatosplenic T-Cell Lymphoma

Search Clinical Trials , NIH Clinical Center for Hepatosplenic T-Cell Lymphoma

Genetic Tests for Hepatosplenic T-Cell Lymphoma

Anatomical Context for Hepatosplenic T-Cell Lymphoma

MalaCards organs/tissues related to Hepatosplenic T-Cell Lymphoma:

41
Spleen, T Cells, Bone, Liver, Bone Marrow, B Cells, Myeloid

Publications for Hepatosplenic T-Cell Lymphoma

Articles related to Hepatosplenic T-Cell Lymphoma:

(show top 50) (show all 119)
# Title Authors Year
1
Hepatosplenic T cell lymphoma: a unifying entity in a patient with hemolytic anemia, massive splenomegaly, and liver dysfunction. ( 29766398 )
2018
2
Intrasinusoidal pattern of bone marrow infiltration by hepatosplenic T-cell lymphoma. ( 29636955 )
2018
3
Expect the unexpected - Loss of surface CD3 on flow cytometry in hepatosplenic T-cell lymphoma: An eye opener. ( 29676377 )
2018
4
Hepatosplenic T-Cell Lymphoma Arising in a Patient Treated With Tumor Necrosis Factor-I+ Inhibitors for Ankylosing Spondylitis. ( 29933321 )
2018
5
Hepatosplenic T-cell Lymphoma: A Review of Clinicopathologic Features, Pathogenesis and Prognostic Factors. ( 29337025 )
2018
6
Loss of CD3 and TCRI^I' expression in a relapsed hepatosplenic T-cell lymphoma. ( 28573906 )
2018
7
Leukemic conversion of hepatosplenic T-cell lymphoma with pleomorphic morphology and an aggressive course. ( 29676383 )
2018
8
Peripheral T-Cell Lymphomas: Incorporating New Developments in Diagnostics, Prognostication, and Treatment Into Clinical Practice-PART 2: ENKTL, EATL, Indolent T-Cell LDP of the GI Tract, ATLL, and Hepatosplenic T-Cell Lymphoma. ( 30153323 )
2018
9
DNA methylation profiling identifies candidate genes for the pathogenesis of hepatosplenic T-cell lymphoma. ( 30337361 )
2018
10
Successful Bridging Chemotherapy with Gemcitabine, Carboplatin, and Dexamethasone before Unrelated Stem Cell Transplantation for Hepatosplenic T-cell Lymphoma. ( 30449784 )
2018
11
Hepatosplenic T-Cell Lymphoma in an Immunocompetent Male with Central Nervous System Invasion: A Rare Clinical Entity. ( 30500103 )
2018
12
The Genetic Basis of Hepatosplenic T-cell Lymphoma. ( 28122867 )
2017
13
Leukemic phase of hepatosplenic T-cell lymphoma: A great masquerader of acute leukemia. ( 28937394 )
2017
14
Incidence, clinical findings, and survival of hepatosplenic T-cell lymphoma in the United States. ( 28263402 )
2017
15
Hepatosplenic T-cell lymphoma arising in patients with immunodysregulatory disorders: a study of 7 patients who did not receive tumor necrosis factor-I+ inhibitor therapy and literature review. ( 28038706 )
2017
16
Hepatosplenic T-cell lymphoma associated with membranoproliferative glomerulonephritis. ( 28351190 )
2017
17
Distinguishing Between Hepatosplenic T-cell Lymphoma and I^I' T-cell Large Granular Lymphocytic Leukemia: A Clinicopathologic, Immunophenotypic, and Molecular Analysis. ( 27755009 )
2017
18
Dyspoietic changes associated with hepatosplenic T-cell lymphoma are not a manifestation of a myelodysplastic syndrome: analysis of 25 patients. ( 26997444 )
2016
19
Hepatosplenic T-Cell Lymphoma Mimicking Acute Myeloid Leukemia. ( 26708981 )
2016
20
Cutaneous presentation of hepatosplenic T-cell lymphoma-a potential mimicker of primary cutaneous gamma-delta T-cell lymphoma. ( 27562705 )
2016
21
Hepatosplenic T-cell lymphoma in a 47-year-old Crohn's disease patient on thiopurine monotherapy. ( 28058028 )
2016
22
Hepatosplenic T-cell lymphoma in a young immunocompetent man. ( 27033291 )
2016
23
Four hepatosplenic T-cell lymphoma cases of Japanese patients. ( 26870659 )
2016
24
Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases. ( 26872013 )
2016
25
Hepatosplenic T-cell lymphoma: an acute leukemia presentation. ( 27158719 )
2016
26
Hepatosplenic T-cell lymphoma with blastoid morphology in a patient with Crohn disease. ( 27811192 )
2016
27
Two Cases of Hepatosplenic T-Cell Lymphoma in Adolescents Treated for Autoimmune Hepatitis. ( 27516526 )
2016
28
Hepatosplenic T-cell lymphoma: an acute leukemia presentation ( 28845955 )
2016
29
Hepatosplenic T Cell Lymphoma in an Immunocompetent Female Diagnosed using Flow Cytometry: A Rare Clinical Entity. ( 27656456 )
2016
30
Gastroenterology: Hepatosplenic T cell lymphoma associated with schistosomiasis. ( 26211690 )
2015
31
Hepatosplenic T-cell lymphoma with aberrant expression of serum I^-HCG: a case report. ( 26108251 )
2015
32
Hepatosplenic T-Cell Lymphoma: A Clinicopathologic Review With an Emphasis on Diagnostic Differentiation From Other T-Cell/Natural Killer-Cell Neoplasms. ( 26317456 )
2015
33
Hepatosplenic T-cell lymphoma with coexistent hepatitis B infection: a rare clinicopathologic entity. ( 26481866 )
2015
34
Childhood Hepatosplenic T-cell Lymphoma with Skin Involvement. ( 26061932 )
2015
35
Outcomes of allogeneic stem cell transplantation in hepatosplenic T-cell lymphoma. ( 26047388 )
2015
36
Hepatosplenic T-cell lymphoma mimicking bone marrow metastasis. ( 26788568 )
2015
37
Hepatosplenic T-Cell Lymphoma: A Population-Based Study Assessing Incidence and Association With Immune-Mediated Disease. ( 27099586 )
2015
38
Hepatosplenic T-cell lymphoma. ( 25221804 )
2014
39
Allogeneic and autologous stem cell transplantation for hepatosplenic T-cell lymphoma: a retrospective study of the EBMT Lymphoma Working Party. ( 25234166 )
2014
40
Lymphoma with features intermediate between aggressive T-large granular lymphocytic leukemia and hepatosplenic T-cell lymphoma: a diagnostic dilemma? ( 24524924 )
2014
41
Simultaneous hepatosplenic T-cell lymphoma and myelofibrosis. ( 24761381 )
2014
42
Successful treatment with reduced-intensity cord blood transplant in a patient with relapsed refractory hepatosplenic T-cell lymphoma. ( 25065703 )
2014
43
De novo CD3 negative hepatosplenic T-cell lymphoma: diagnostic challenges and pitfalls. ( 24978925 )
2014
44
Hepatosplenic T-cell lymphoma: A case series. ( 25450840 )
2014
45
Integrative genomic and transcriptomic analysis identified candidate genes implicated in the pathogenesis of hepatosplenic T-cell lymphoma. ( 25057852 )
2014
46
Hepatosplenic T cell lymphoma: a rare case report. ( 25332523 )
2014
47
Hepatosplenic T cell lymphoma. ( 25332524 )
2014
48
Purpura of the face and neck: an atypical clinical presentation revealing a hepatosplenic T cell lymphoma. ( 24707248 )
2014
49
Hepatosplenic T-cell lymphoma: a rare cause of hepatosplenomegaly. ( 25012882 )
2014
50
Coexistent hairy cell leukaemia and hepatosplenic T-cell lymphoma: a case report. ( 24621139 )
2014

Variations for Hepatosplenic T-Cell Lymphoma

Expression for Hepatosplenic T-Cell Lymphoma

LifeMap Discovery
Genes differentially expressed in tissues of Hepatosplenic T-Cell Lymphoma patients vs. healthy controls: 35 (show top 50) (show all 400)
# Gene Description Tissue Up/Dn Fold Change (log2) P value
1 TMEM178A transmembrane protein 178A Spleen + 9.71 0.000
2 LPAR3 lysophosphatidic acid receptor 3 Spleen + 8.91 0.000
3 SOX2 SRY (sex determining region Y)-box 2 Spleen + 8.60 0.000
4 MLC1 megalencephalic leukoencephalopathy with subcortical cysts 1 Spleen + 7.68 0.001
5 CLEC3B C-type lectin domain family 3, member B Spleen - 7.16 0.001
6 KIR2DS1 killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 1 Spleen + 7.16 0.000
7 HLA-DQA1 major histocompatibility complex, class II, DQ alpha 1 Spleen - 7.01 0.023
8 KIR3DL1 killer cell immunoglobulin-like receptor, three domains, long cytoplasmic tail, 1 Spleen + 6.95 0.000
9 KIR3DS1 killer cell immunoglobulin-like receptor, three domains, short cytoplasmic tail, 1 Spleen + 6.85 0.001
10 ADAMTS17 ADAM metallopeptidase with thrombospondin type 1 motif, 17 Spleen + 6.83 0.000
11 KIR3DL3 killer cell immunoglobulin-like receptor, three domains, long cytoplasmic tail, 3 Spleen + 6.66 0.000
12 KIR2DL3 killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 3 Spleen + 6.63 0.001
13 MARCO macrophage receptor with collagenous structure Spleen + 6.61 0.009
14 KIR2DS3 killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 3 Spleen + 6.34 0.001
15 CXCL13 chemokine (C-X-C motif) ligand 13 Spleen - 6.26 0.004
16 OLFML2A olfactomedin-like 2A Spleen - 6.25 0.015
17 KIR2DL2 killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 2 Spleen + 6.21 0.002
18 KIR2DS2 killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 2 Spleen + 6.14 0.000
19 KIR2DS4 killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 4 Spleen + 6.13 0.001
20 GREB1 growth regulation by estrogen in breast cancer 1 Spleen + 6.09 0.001
21 KIR2DL1 killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 1 Spleen + 6.02 0.002
22 LOC100505501 uncharacterized LOC100505501 Spleen + 5.96 0.000
23 MAATS1 MYCBP-associated, testis expressed 1 Spleen - 5.93 0.000
24 TRGV5 T cell receptor gamma variable 5 Spleen + 5.90 0.000
25 CRHBP corticotropin releasing hormone binding protein Spleen - 5.89 0.000
26 KLRC3 killer cell lectin-like receptor subfamily C, member 3 Spleen + 5.87 0.000
27 SMIM24 small integral membrane protein 24 Spleen + 5.85 0.000
28 CCM2L cerebral cavernous malformation 2-like Spleen - 5.83 0.005
29 RUNDC3B RUN domain containing 3B Spleen + 5.82 0.000
30 HLA-DQB1 major histocompatibility complex, class II, DQ beta 1 Spleen + 5.80 0.000
31 PCDH7 protocadherin 7 Spleen - 5.78 0.010
32 APLNR apelin receptor Spleen - 5.76 0.000
33 KIR2DL5A killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 5A Spleen + 5.73 0.001
34 KIR2DS5 killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 5 Spleen + 5.69 0.001
35 GIPC2 GIPC PDZ domain containing family, member 2 Spleen - 5.69 0.000
36 GTSF1 gametocyte specific factor 1 Spleen + 5.62 0.001
37 GPM6A glycoprotein M6A Spleen - 5.62 0.000
38 IGFBP3 insulin-like growth factor binding protein 3 Spleen - 5.61 0.004
39 BMP7 bone morphogenetic protein 7 Spleen + 5.59 0.000
40 PDE2A phosphodiesterase 2A, cGMP-stimulated Spleen - 5.58 0.002
41 KIF14 kinesin family member 14 Spleen + 5.55 0.000
42 HYAL1 hyaluronoglucosaminidase 1 Spleen - 5.51 0.006
43 GPR82 G protein-coupled receptor 82 Spleen + 5.49 0.000
44 DNASE1L3 deoxyribonuclease I-like 3 Spleen - 5.44 0.003
45 USP44 ubiquitin specific peptidase 44 Spleen + 5.44 0.000
46 CD96 CD96 molecule Spleen + 5.42 0.001
47 AKAP5 A kinase (PRKA) anchor protein 5 Spleen + 5.41 0.000
48 RNASE1 ribonuclease, RNase A family, 1 (pancreatic) Spleen - 5.40 0.008
49 PLVAP plasmalemma vesicle associated protein Spleen - 5.38 0.001
50 NEK2 NIMA-related kinase 2 Spleen + 5.35 0.000
Search GEO for disease gene expression data for Hepatosplenic T-Cell Lymphoma.

Pathways for Hepatosplenic T-Cell Lymphoma

GO Terms for Hepatosplenic T-Cell Lymphoma

Biological processes related to Hepatosplenic T-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 T cell activation GO:0042110 8.8 CD7 DPP4 PIK3CD

Molecular functions related to Hepatosplenic T-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 alpha-tubulin binding GO:0043014 8.62 INO80 SETD2

Sources for Hepatosplenic T-Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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