NLPHL
MCID: NDL021
MIFTS: 44

Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) malady

Cancer, Blood, Immune categories

Summaries for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
33MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
MalaCards: Nodular Lymphocyte Predominant Hodgkin Lymphoma, also known as NLPHL, is related to lymphoma, diffuse large cell and follicular lymphoma, and has symptoms including lymphadenopathy/polyadenopathies, immunodeficiency/increased susceptibility to infections/recurrent infections and lymphoma. An important gene associated with Nodular Lymphocyte Predominant Hodgkin Lymphoma is PDCD1 (programmed cell death 1), and among its related pathways are Atypical NF-kappaB pathway and NF-kappaB Signaling. The compounds monochloroacetic acid and manganese superoxide have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, lymph node and small intestine, and related mouse phenotypes are skeleton and growth/size.

Aliases & Classifications for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
49Orphanet, 61UMLS, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 25ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer
Anatomical: Blood, Immune


Characteristics (Orphanet epidemiological data):

49
nodular lymphocyte predominant hodgkin lymphoma:
Age of onset: Variable; Age of death: Any age


Aliases & Descriptions:

nodular lymphocyte predominant hodgkin lymphoma 49 61
nlphl 49


External Ids:

ICD10 via Orphanet26 C81.1
SNOMED-CT via Orphanet58 118605002, 277608004, 70600005
UMLS via Orphanet62 C1334968, C2239290
ICD1025 C81.0

Related Diseases for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Nodular Lymphocyte Predominant Hodgkin Lymphoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 66)
idRelated DiseaseScoreTop Affiliating Genes
1lymphoma, diffuse large cell31.0TNFRSF8, PAX5, PTPRC, BCL6
2follicular lymphoma30.8REL, PAX5, PTPRC, BCL6
3bone lymphoma10.5
4lung lymphoma10.4
5autoimmune hemolytic anemia10.4
6kaposi's sarcoma10.4
7pediatric lymphoma10.4
8adult lymphoma10.4
9n syndrome10.4
10hermansky-pudlak syndrome10.4
11angioimmunoblastic t-cell lymphoma10.0BCL6
12diffuse large b-cell lymphoma10.0MUM1
13testicular lymphoma10.0BCL6
14histiocytosis10.0CD68
15central nervous system lymphoma10.0BCL6
16cutaneous fibrous histiocytoma10.0CD68
17myeloid sarcoma10.0FUT4
18seminoma10.0TNFRSF8
19systemic lupus erythematosus10.0TNFAIP3, PDCD1
20myeloid leukemia10.0PTPRC
21herpes simplex10.0CFLAR, POU2F2
22lymphosarcoma10.0TNFRSF8, BCL6
23arthritis10.0NFKBIA, CFLAR
24adenoma10.0TNFRSF8
25sarcoidosis10.0PTPRC
26hepatitis c10.0PAX5
27myelodysplastic syndromes10.0CFLAR, NFKBIA
28atherosclerosis10.0CD68, NFKBIA
29malignant histiocytosis10.0TNFRSF8
30colorectal cancer10.0CFLAR, NFKBIA
31reticulosarcoma10.0BCL6, TNFRSF8, TIA1
32retinoblastoma10.0PTPRC, CARD16
33central nervous system lymphoma, primary10.0BCL6, PAX5
34angiomyolipoma10.0VIM, PTPRC
35lymphomatoid papulosis10.0TNFRSF8, TIA1
36nephrotic syndrome10.0NFKBIA, PTPRC
37tuberculosis10.0PTPRC, TNFRSF8
38sarcoma10.0VIM, CFLAR, PTPRC
39mycosis fungoides10.0TIA1, TNFRSF8
40acute leukemia10.0PTPRC, PAX5
41multiple sclerosis10.0PDCD1, NFKBIA, CFLAR, PTPRC
42giant cell tumor10.0VIM, TNFRSF8, PTPRC
43lymphoblastic lymphoma10.0PAX5, PTPRC, BCL6
44ovarian cancer10.0CFLAR, REL, NFKBIA
45peripheral primitive neuroectodermal tumor10.0PTPRC, CFLAR, TNFRSF8, VIM
46malignant fibroxanthoma10.0VIM, TNFRSF8, CD68, PTPRC
47immunodeficiency with hyper igm type 210.0TNFRSF8, NFKBIA, PTPRC, BCL6
48b-cell chronic lymphocytic leukemia10.0TNFRSF8, NFKBIA, PAX5, BCL6
49anaplastic large cell lymphoma10.0TNFRSF8, PAX5, PTPRC, BCL6
50breast cancer10.0BCL6, CFLAR, REL, NFKBIA, TNFAIP3, TNFRSF8

Graphical network of the top 20 diseases related to Nodular Lymphocyte Predominant Hodgkin Lymphoma:



Diseases related to nodular lymphocyte predominant hodgkin lymphoma

Clinical Features for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
49Orphanet
See all sources


Symptoms:

49 (show all 13)
  • lymphadenopathy/polyadenopathies
  • immunodeficiency/increased susceptibility to infections/recurrent infections
  • lymphoma
  • hyperhidrosis/increased sweating
  • pruritus/itching
  • hepatomegaly/liver enlargement (excluding storage disease)
  • splenomegaly
  • anorexia
  • bone marrow failure/pancytopenia
  • neoplasms/tumors
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • fever/chilling
  • asthenia/fatigue/weakness

Drugs & Therapeutics for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
See all sources

Approved drugs:

Search CenterWatch for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Drug clinical trials:

Search ClinicalTrials for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Search NIH Clinical Center for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Search CenterWatch for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Genetic Tests for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Anatomical Context for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Nodular Lymphocyte Predominant Hodgkin Lymphoma:

33
Bone marrow, Lymph node, Small intestine, Lung, Cervix, Monocytes, T cells, B cells

Animal Models for Nodular Lymphocyte Predominant Hodgkin Lymphoma or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Nodular Lymphocyte Predominant Hodgkin Lymphoma:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053909.1PTPRC, PAX5, CD68, PDCD1, TIA1
2MP:00053786.6VIM, TIA1, TNFAIP3, NFKBIA, POU2AF1, POU2F2
3MP:00107686.2NFKBIA, TNFAIP3, PDCD1, TIA1, VIM, POU2AF1
4MP:00053845.9VIM, PDCD1, TNFAIP3, NFKBIA, POU2AF1, POU2F2
5MP:00053975.1POU2AF1, NFKBIA, TNFAIP3, TNFRSF8, PDCD1, TIA1
6MP:00053874.8BCL6, VIM, TIA1, PDCD1, TNFRSF8, TNFAIP3

Publications for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
51PubMed
See all sources

Articles related to Nodular Lymphocyte Predominant Hodgkin Lymphoma:

(show top 50)    (show all 83)
idTitleAuthorsYear
1
High-dose chemotherapy and autologous stem cell transplantation for nodular lymphocyte-predominant Hodgkin lymphoma. (23507470)
2013
2
Does functional imaging distinguish nodular lymphocyte-predominant hodgkin lymphoma from T-cell/histiocyte-rich large B-cell lymphoma? (23773450)
2013
3
Occurrence of nodular lymphocyte-predominant hodgkin lymphoma in hermansky-pudlak type 2 syndrome is associated to natural killer and natural killer T cell defects. (24302998)
2013
4
VII. Management of nodular lymphocyte-predominant Hodgkin lymphoma. (23775649)
2013
5
High familial risk in nodular lymphocyte-predominant Hodgkin lymphoma. (23284040)
2013
6
How I treat nodular lymphocyte predominant Hodgkin lymphoma. (24215035)
2013
7
Primary nodular lymphocyte predominant Hodgkin lymphoma of the palate: a rare incidence which was also associated with progressive transformation of germinal centres of cervical lymph node. (23932753)
2013
8
Failure of fluorodeoxyglucose positron emission tomography to detect the high-grade transformation of nodular lymphocyte predominant Hodgkin's lymphoma. (23328622)
2013
9
Looking for familial nodular lymphocyte-predominant Hodgkin lymphoma. (23686933)
2013
10
Autoimmune hemolytic anemia and nodular lymphocyte-predominant hodgkin lymphoma: a rare association. (23710384)
2013
11
Utility of LRF/Pokemon and NOTCH1 Protein Expression in the Distinction of Nodular Lymphocyte-Predominant Hodgkin Lymphoma and Classical Hodgkin Lymphoma. (24326827)
2013
12
Intranodular clusters of activated cells with T follicular helper phenotype in nodular lymphocyte predominant Hodgkin lymphoma: a pilot study of 32 cases from Finland. (23684509)
2013
13
Nodular lymphocyte predominant Hodgkin's lymphoma of the cervix: A case report of a rare entity. (24371660)
2012
14
Rituximab plus Ifosfamide, Carboplatin and Etoposide for T-Cell/Histiocyte-Rich B-Cell Lymphoma Arising in Nodular Lymphocyte-Predominant Hodgkin's Lymphoma. (22949903)
2012
15
Nodular lymphocyte-predominant Hodgkin lymphoma: cytopathologic correlates on fine-needle aspiration. (22367911)
2012
16
Nodular lymphocyte predominant Hodgkin lymphoma in children and adolescents--a comprehensive review of biology, clinical course and treatment options. (22994199)
2012
17
Nodular lymphocyte-predominant hodgkin lymphoma with atypical T cells: a morphologic variant mimicking peripheral T-cell lymphoma. (21997687)
2011
18
Mycobacterium avium intracellulare infection coexistent with nodular lymphocyte predominant Hodgkin lymphoma involving the lung. (21399527)
2011
19
Coexisting and clonally identical classic hodgkin lymphoma and nodular lymphocyte predominant hodgkin lymphoma. (21490448)
2011
20
Nodular lymphocyte predominant Hodgkin lymphoma behaves as a distinct clinical entity with good outcome: evidence from 14-year follow-up in the West of Scotland Cancer Network. (21663507)
2011
21
Analysis of KLHDC8B in familial nodular lymphocyte predominant Hodgkin lymphoma. (21517803)
2011
22
Phase 2 study of rituximab in newly diagnosed stage IA nodular lymphocyte-predominant Hodgkin lymphoma: a report from the German Hodgkin Study Group. (21828141)
2011
23
Nodular lymphocyte predominant Hodgkin lymphoma and diffuse large B-cell lymphoma: a study of six cases concurrently involving the same site. (22175899)
2011
24
CD15-expressing nodular lymphocyte-predominant Hodgkin lymphoma. (21457163)
2011
25
Nodular, lymphocyte-predominant Hodgkin lymphoma: a long-term study and analysis of transformation to diffuse large B-cell lymphoma in a cohort of 164 patients from the Adult Lymphoma Study Group. (20029973)
2010
26
Nodular lymphocyte predominant Hodgkin lymphoma: diagnostic pearls and pitfalls. (20923296)
2010
27
Nodular lymphocyte-predominant Hodgkin lymphoma and T cell histiocyte-rich large B cell lymphoma: diagnosis in two monozygotic twins. (20653789)
2010
28
Febrile cholestatic disease as an initial presentation of nodular lymphocyte-predominant Hodgkin lymphoma. (20845519)
2010
29
Mutations in the genes coding for the NF-I_B regulating factors II_BI+ and A20 are uncommon in nodular lymphocyte-predominant Hodgkin's lymphoma. (19648161)
2010
30
Nodular lymphocyte predominant Hodgkin lymphoma with clusters of LP Cells, acute inflammation, and fibrosis: a syncytial variant. (19730363)
2009
31
Nodular lymphocyte predominant Hodgkin lymphoma. (19605845)
2009
32
Refractory nodular lymphocyte predominant Hodgkin lymphoma transformed to T-cell/histiocyte-rich B-cell lymphoma in an adolescent: salvage therapy with allogeneic bone marrow transplantation. (19131792)
2008
33
Nodular lymphocyte-predominant Hodgkin lymphoma presenting as fulminant hepatic failure in a pediatric patient: a case report with pathologic, immunophenotypic, and molecular findings. (18227720)
2008
34
Nodular lymphocyte predominant Hodgkin lymphoma at atypical locations may be associated with increased numbers of large cells and a diffuse histologic component. (17918256)
2008
35
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL)-clinicopathological features based on the data of two Hungarian lymphoma centres. (18431694)
2008
36
Nodular lymphocyte-predominant hodgkin lymphoma or T-cell/histiocyte rich large B-cell lymphoma: the problem in "grey zone" lymphomas. (18784812)
2008
37
Origin and pathogenesis of nodular lymphocyte-predominant Hodgkin lymphoma as revealed by global gene expression analysis. (18794340)
2008
38
Bilateral proptosis as the initial presentation of systemic nodular lymphocyte predominant Hodgkin lymphoma. (17613778)
2007
39
Nodular lymphocyte-predominant hodgkin lymphoma. (17591565)
2007
40
Anti-CD20 monoclonal antibody (rituximab) for therapy of CD20-positive nodular lymphocyte-predominant Hodgkin lymphoma in an 10-year-old girl. (17065142)
2006
41
IgD positive L&H cells identify a unique subset of nodular lymphocyte predominant Hodgkin lymphoma. (16699312)
2006
42
A double-positive CD4+CD8+ T-cell population is commonly found in nodular lymphocyte predominant Hodgkin lymphoma. (17050078)
2006
43
Aberrant somatic hypermutation in tumor cells of nodular-lymphocyte-predominant and classic Hodgkin lymphoma. (16614247)
2006
44
Biology, clinical course and management of nodular lymphocyte-predominant hodgkin lymphoma. (17124071)
2006
45
Familial nodular lymphocyte predominant Hodgkin lymphoma: Successful treatment with CHOP plus rituximab. (16236615)
2005
46
Differential expression patterns of c-REL protein in classic and nodular lymphocyte predominant Hodgkin lymphoma. (15551733)
2004
47
Nodular lymphocyte predominant Hodgkin lymphoma in siblings. (15160926)
2004
48
Characterization of variant patterns of nodular lymphocyte predominant hodgkin lymphoma with immunohistologic and clinical correlation. (14508396)
2003
49
Nodular lymphocyte predominant Hodgkin lymphoma. An immunophenotypic reappraisal based on a single-institution experience. (12579988)
2003
50
Further phenotypic evidence that nodular, lymphocyte-predominant Hodgkin's disease is a large B-cell lymphoma in evolution. (2240355)
1990

Genetic Variations for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Expression for genes affiliated with Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Nodular Lymphocyte Predominant Hodgkin Lymphoma

Search GEO for disease gene expression data for Nodular Lymphocyte Predominant Hodgkin Lymphoma.

Pathways for genes affiliated with Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
38NCBI BioSystems Database, 4Cell Signaling Technology, 52QIAGEN, 12EMD Millipore, 30KEGG
See all sources

Pathways related to Nodular Lymphocyte Predominant Hodgkin Lymphoma according to GeneCards/GeneDecks:

(show all 14)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.0REL, NFKBIA
29.8REL, NFKBIA, TNFAIP3
3
Hide members
9.8REL, NFKBIA, TNFAIP3
4
Hide members
9.8TNFAIP3, NFKBIA, REL
5
Hide members
9.8CFLAR, NFKBIA, TNFAIP3
69.8TNFAIP3, NFKBIA, CFLAR
79.8TNFAIP3, NFKBIA, CFLAR
89.8PTPRC, REL, NFKBIA
9
Apoptosis and survival Anti-apoptotic TNFs/NF-kB/Bcl-2 pathway
Hide members
9.5TNFRSF8, NFKBIA, REL
10
Hide members
9.4NFKBIA, REL, PTPRC, BCL6
119.4BCL6, PTPRC, REL, NFKBIA
129.4NFKBIA, POU2F2, REL
139.0TNFRSF8, TNFAIP3, NFKBIA, REL, CFLAR
14
Hide members
8.6PTPRC, REL, POU2F2, NFKBIA, PDCD1, VIM

Compounds for genes affiliated with Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 60Tocris Bioscience, 24HMDB, 29IUPHAR
See all sources

Compounds related to Nodular Lymphocyte Predominant Hodgkin Lymphoma according to GeneCards/GeneDecks:

(show all 23)
idCompoundScoreTop Affiliating Genes
1monochloroacetic acid4510.2VIM, PTPRC
2manganese superoxide4510.1REL, NFKBIA, TNFAIP3
3dutp4510.0CARD16, NFKBIA, CFLAR
4toluidine4510.0VIM, PTPRC
5rituximab45 50 1111.9BCL6, PAX5, CFLAR
6serine459.7NFKBIA, POU2F2, CFLAR
7etoposide45 50 60 1112.6VIM, CARD16, NFKBIA, CFLAR
8vincristine45 50 1111.6BCL6, CFLAR, VIM
912-o-tetradecanoylphorbol 13-acetate459.5VIM, NFKBIA, PTPRC, BCL6
10[alcl]459.5TIA1, TNFRSF8, PAX5, PTPRC
11estradiol45 11 2411.4PAX5, CFLAR, NFKBIA, VIM
12adriamycin459.4CFLAR, NFKBIA, VIM
13h2o2459.4VIM, TNFAIP3, NFKBIA, CFLAR, PTPRC
14cyclosporin a45 29 6011.3PTPRC, CFLAR, REL, NFKBIA, TNFRSF8
15testosterone45 60 11 2412.3PTPRC, PAX5, CFLAR, NFKBIA, VIM
16doxorubicin45 50 1111.3VIM, NFKBIA, CFLAR, BCL6
17cysteine459.1VIM, CARD16, NFKBIA, REL, CFLAR, PTPRC
18threonine459.1TIA1, TNFRSF8, NFKBIA, REL, CFLAR, PTPRC
19retinoic acid45 249.9VIM, NFKBIA, REL, CFLAR, FUT4, BCL6
20oligonucleotide458.9VIM, NFKBIA, POU2F2, REL, CFLAR, PAX5
21paraffin458.4VIM, TIA1, TNFRSF8, CD68, PAX5, PTPRC
22lipid458.1VIM, CARD16, TNFRSF8, NFKBIA, SWAP70, CD68
23tyrosine456.4BCL6, CARD16, PDCD1, TNFRSF8, NFKBIA, POU2AF1

GO Terms for genes affiliated with Nodular Lymphocyte Predominant Hodgkin Lymphoma

Sources:
16Gene Ontology
See all sources

Biological processes related to Nodular Lymphocyte Predominant Hodgkin Lymphoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1negative regulation of extrinsic apoptotic signaling pathway via death domain receptorsGO:190204210.0CFLAR, TNFAIP3
2regulation of germinal center formationGO:0026349.8BCL6, TNFAIP3
3response to muramyl dipeptideGO:0324959.7NFKBIA, TNFAIP3
4apoptotic processGO:0069159.0NFKBIA, TNFAIP3, CFLAR, TIA1, VIM, PDCD1
5humoral immune responseGO:0069598.6PAX5, POU2AF1, POU2F2, PDCD1

Products for genes affiliated with Nodular Lymphocyte Predominant Hodgkin Lymphoma

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Nodular Lymphocyte Predominant Hodgkin Lymphoma

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet