NLPHL
MCID: NDL021
MIFTS: 46

Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) malady

Cancer diseases, Blood diseases, Rare diseases, Immune diseases categories
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Summaries for Nodular Lymphocyte Predominant Hodgkin Lymphoma

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MalaCards based summary: Nodular Lymphocyte Predominant Hodgkin Lymphoma, also known as NLPHL, is related to diffuse large b-cell lymphoma and peripheral t-cell 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 Development Glucocorticoid receptor signaling and B Cell Receptor Signaling Pathway. The compounds cytidine and rituximab have been mentioned in the context of this disorder. Affiliated tissues include t cells, b cells and bone marrow, and related mouse phenotypes are growth/size/body and cellular.

Aliases & Classifications for Nodular Lymphocyte Predominant Hodgkin Lymphoma

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Sources:
48Orphanet, 62UMLS, 26ICD10 via Orphanet, 63UMLS via Orphanet, 25ICD10
See all sources

Nodular Lymphocyte Predominant Hodgkin Lymphoma, Aliases & Descriptions:

Name: Nodular Lymphocyte Predominant Hodgkin Lymphoma 48 62
 
Nlphl 48 62


Classifications:

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


Characteristics (Orphanet epidemiological data):

48
nodular lymphocyte predominant hodgkin lymphoma:
Age of onset: Variable; Age of death: Any age


External Ids:

ICD10 via Orphanet26 C81.1
UMLS via Orphanet63 C1334968, C2239290
ICD1025 C81.0

Related Diseases for Nodular Lymphocyte Predominant Hodgkin Lymphoma

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



Diseases related to nodular lymphocyte predominant hodgkin lymphoma

Symptoms for Nodular Lymphocyte Predominant Hodgkin Lymphoma

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Symptoms:

48 (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

HPO human phenotypes related to Nodular Lymphocyte Predominant Hodgkin Lymphoma:

(show all 11)
id Description Frequency HPO Source Accession
1 lymphoma hallmark (90%) HP:0002665
2 lymphadenopathy hallmark (90%) HP:0002716
3 abnormality of immune system physiology hallmark (90%) HP:0010978
4 hyperhidrosis occasional (7.5%) HP:0000975
5 pruritus occasional (7.5%) HP:0000989
6 splenomegaly occasional (7.5%) HP:0001744
7 weight loss occasional (7.5%) HP:0001824
8 anorexia occasional (7.5%) HP:0002039
9 hepatomegaly occasional (7.5%) HP:0002240
10 abnormality of temperature regulation occasional (7.5%) HP:0004370
11 bone marrow hypocellularity occasional (7.5%) HP:0005528

Drugs & Therapeutics for Nodular Lymphocyte Predominant Hodgkin Lymphoma

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Drug clinical trials:

Search ClinicalTrials for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Search NIH Clinical Center for Nodular Lymphocyte Predominant Hodgkin Lymphoma

Genetic Tests for Nodular Lymphocyte Predominant Hodgkin Lymphoma

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Anatomical Context for Nodular Lymphocyte Predominant Hodgkin Lymphoma

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

32
T cells, B cells, Bone marrow, Bone, Liver, Lymph node, Small intestine, Lung, Monocytes

Animal Models for Nodular Lymphocyte Predominant Hodgkin Lymphoma or affiliated genes

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MGI Mouse Phenotypes related to Nodular Lymphocyte Predominant Hodgkin Lymphoma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053787.7POU2AF1, BCL6, REL, PAX5, POU2F2
2MP:00053847.4POU2AF1, REL, BCL6, PDCD1, SWAP70, PAX5
3MP:00107687.1POU2AF1, PDCD1, BCL6, REL, PAX5, POU2F2
4MP:00053976.9POU2AF1, SWAP70, PDCD1, BCL6, REL, PAX5
5MP:00053876.9SWAP70, REL, PAX5, POU2F2, POU2AF1, PDCD1

Publications for Nodular Lymphocyte Predominant Hodgkin Lymphoma

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Articles related to Nodular Lymphocyte Predominant Hodgkin Lymphoma:

(show top 50)    (show all 89)
idTitleAuthorsYear
1
Nodular lymphocyte predominant hodgkin lymphoma: biology, diagnosis and treatment. (24650975)
2014
2
Performance of FDG PET/CT at initial diagnosis in a rare lymphoma: nodular lymphocyte-predominant Hodgkin lymphoma. (24965842)
2014
3
Primary adrenal nodular lymphocyte-predominant Hodgkin lymphoma: A case report and review of the literature. (25120675)
2014
4
Intrafollicular classical Hodgkin lymphoma mimicking nodular lymphocyte predominant Hodgkin lymphoma: A report of two cases. (25394195)
2014
5
Nodular lymphocyte-predominant Hodgkin lymphoma in children: clinical course, biology, and management. (25296072)
2014
6
EBV may be expressed in the LP cells of nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) in both children and adults. (24525501)
2014
7
Open questions in the management of nodular lymphocyte predominant hodgkin lymphoma. (24772357)
2014
8
Fluorescence immunophenotyping and interphase cytogenetics (FICTION) detects BCL6 abnormalities, including gene amplification, in most cases of nodular lymphocyte-predominant Hodgkin lymphoma. (24678684)
2014
9
The prognostic impact of variant histology in nodular lymphocyte-predominant Hodgkin lymphoma: a report from the German Hodgkin Study Group (GHSG). (24100447)
2013
10
Treatment of nodular lymphocyte-predominant Hodgkin lymphoma-"do no harm". (24472810)
2013
11
High-dose chemotherapy and autologous stem cell transplantation for nodular lymphocyte-predominant Hodgkin lymphoma. (23507470)
2013
12
Nodular lymphocyte predominant hodgkin lymphoma and T cell/histiocyte rich large B cell lymphoma - endpoints of a spectrum of one disease? (24244368)
2013
13
Does functional imaging distinguish nodular lymphocyte-predominant hodgkin lymphoma from T-cell/histiocyte-rich large B-cell lymphoma? (23773450)
2013
14
Complete response to brentuximab vedotin in a transplant-naA^ve patient with relapsed CD30-positive nodular lymphocyte-predominant Hodgkin lymphoma. (24472809)
2013
15
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
16
VII. Management of nodular lymphocyte-predominant Hodgkin lymphoma. (23775649)
2013
17
High familial risk in nodular lymphocyte-predominant Hodgkin lymphoma. (23284040)
2013
18
Does "in situ lymphoma" occur as a distinct step in the development of nodular lymphocyte-predominant Hodgkin lymphoma? (21732334)
2012
19
The unique entity of nodular lymphocyte-predominant Hodgkin lymphoma: current approaches to diagnosis and management. (21812538)
2012
20
"Intrafollicular neoplasia" of nodular lymphocyte predominant Hodgkin lymphoma: description of a hypothetic early step of the disease. (22209342)
2012
21
Peripheral blood lymphocyte/monocyte ratio at diagnosis and survival in nodular lymphocyte-predominant Hodgkin lymphoma. (22360654)
2012
22
Nodular lymphocyte-predominant hodgkin lymphoma with atypical T cells: a morphologic variant mimicking peripheral T-cell lymphoma. (21997687)
2011
23
Mycobacterium avium intracellulare infection coexistent with nodular lymphocyte predominant Hodgkin lymphoma involving the lung. (21399527)
2011
24
Coexisting and clonally identical classic hodgkin lymphoma and nodular lymphocyte predominant hodgkin lymphoma. (21490448)
2011
25
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
26
Analysis of KLHDC8B in familial nodular lymphocyte predominant Hodgkin lymphoma. (21517803)
2011
27
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
28
Programmed death 1 expression in variant immunoarchitectural patterns of nodular lymphocyte predominant Hodgkin lymphoma: comparison with CD57 and lymphomas in the differential diagnosis. (20825974)
2010
29
Nodular lymphocyte predominant Hodgkin lymphoma: diagnostic pearls and pitfalls. (20923296)
2010
30
Nodular lymphocyte-predominant Hodgkin lymphoma and T cell histiocyte-rich large B cell lymphoma: diagnosis in two monozygotic twins. (20653789)
2010
31
Concurrent occurrence of nodular lymphocyte predominant Hodgkin lymphoma and Kaposi sarcoma in lymph nodes: a first case report. (20595178)
2010
32
Nodular lymphocyte predominant Hodgkin lymphoma with clusters of LP Cells, acute inflammation, and fibrosis: a syncytial variant. (19730363)
2009
33
The emerging role for rituximab in the treatment of nodular lymphocyte predominant Hodgkin lymphoma. (19606035)
2009
34
Paraneoplastic lumbosacral axonal polyradiculopathy preceding the diagnosis of nodular lymphocyte predominant Hodgkin lymphoma: a case report. (18720211)
2008
35
Beyond the lymphocyte predominant cell: CD4+CD8+ T-cells in nodular lymphocyte predominant Hodgkin lymphoma. (18949610)
2008
36
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
37
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
38
Nodular lymphocyte predominant Hodgkin lymphoma at atypical locations may be associated with increased numbers of large cells and a diffuse histologic component. (17918256)
2008
39
Outcome of children with nodular lymphocyte predominant Hodgkin lymphoma - a Children's Cancer and Leukaemia Group report. (17760808)
2007
40
Bilateral proptosis as the initial presentation of systemic nodular lymphocyte predominant Hodgkin lymphoma. (17613778)
2007
41
Anti-CD20 monoclonal antibody (rituximab) for therapy of CD20-positive nodular lymphocyte-predominant Hodgkin lymphoma in an 10-year-old girl. (17065142)
2006
42
IgD positive L&H cells identify a unique subset of nodular lymphocyte predominant Hodgkin lymphoma. (16699312)
2006
43
A double-positive CD4+CD8+ T-cell population is commonly found in nodular lymphocyte predominant Hodgkin lymphoma. (17050078)
2006
44
Familial nodular lymphocyte predominant Hodgkin lymphoma: Successful treatment with CHOP plus rituximab. (16236615)
2005
45
Differential expression patterns of c-REL protein in classic and nodular lymphocyte predominant Hodgkin lymphoma. (15551733)
2004
46
Nodular lymphocyte predominant Hodgkin lymphoma in siblings. (15160926)
2004
47
Bone marrow involvement in patients with nodular lymphocyte predominant Hodgkin lymphoma. (15087668)
2004
48
Diffuse large B-cell lymphoma arising in nodular lymphocyte predominant hodgkin lymphoma. A report of 21 cases from the Nebraska Lymphoma Study Group. (14738141)
2003
49
Characterization of variant patterns of nodular lymphocyte predominant hodgkin lymphoma with immunohistologic and clinical correlation. (14508396)
2003
50
Frequent occurrence of BCL6 rearrangements in nodular lymphocyte predominance Hodgkin lymphoma but not in classical Hodgkin lymphoma. (12393409)
2003

Variations for Nodular Lymphocyte Predominant Hodgkin Lymphoma

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Expression for genes affiliated with Nodular Lymphocyte Predominant Hodgkin Lymphoma

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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

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

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5POU2F2, REL
29.3BCL6, REL
39.2BCL6, PAX5
4
Show member pathways
BCR signaling pathway37
TCR Signaling Pathway37
9.1PDCD1, REL, POU2F2
58.9BCL6, REL, PAX5

Compounds for genes affiliated with Nodular Lymphocyte Predominant Hodgkin Lymphoma

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

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

idCompoundScoreTop Affiliating Genes
1cytidine44 24 1111.3BCL6, PAX5
2rituximab44 50 1111.2PAX5, BCL6
3zinc44 249.9BCL6, REL, PAX5
4oligonucleotide448.8POU2F2, PAX5, REL
5serine448.1BCL6, REL, PAX5, POU2F2
6tyrosine447.0SWAP70, POU2AF1, POU2F2, PAX5, REL, BCL6

GO Terms for genes affiliated with Nodular Lymphocyte Predominant Hodgkin Lymphoma

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

idNameGO IDScoreTop Affiliating Genes
1nucleusGO:0056347.1SWAP70, BCL6, MUM1, PAX5, POU2F2, POU2AF1

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

idNameGO IDScoreTop Affiliating Genes
1transcription from RNA polymerase II promoterGO:0063668.9PAX5, POU2F2, POU2AF1
2humoral immune responseGO:0069598.3PDCD1, PAX5, POU2F2, POU2AF1

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

idNameGO IDScoreTop Affiliating Genes
1sequence-specific DNA bindingGO:0435658.7BCL6, REL, POU2F2
2sequence-specific DNA binding transcription factor activityGO:0037007.9BCL6, REL, PAX5, POU2F2

Products for genes affiliated with Nodular Lymphocyte Predominant Hodgkin Lymphoma

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  • Antibodies
  • Proteins
  • Lysates

Sources for Nodular Lymphocyte Predominant Hodgkin Lymphoma

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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