MCID: GMM003
MIFTS: 30

Gamma Heavy Chain Disease malady

Blood, Cancer categories

Summaries for Gamma Heavy Chain Disease

Sources:
8Disease Ontology, 43NIH Rare Diseases, 33MalaCards
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NIH Rare Diseases:43 Gamma heavy chain disease is characterized by the abnormal production of antibodies. antibodies are made up of light chains and heavy chains. in this disorder, the heavy chain of the gamma antibody (igg) is overproduced by the body.  gamma heavy chain disease mainly affects older adults and is similar to aggressive malignant (cancerous) lymphoma. however, some people with this disorder have no symptoms. people with symptoms may respond to chemotherapy drugs, corticosteroids, and radiation therapy. approximately one-third of individuals with gamma heavy chain disease are also diagnosed with an autoimmune disorder. last updated: 7/25/2010

MalaCards: Gamma Heavy Chain Disease, also known as franklin disease, is related to heavy chain deposition disease and splenomegaly, and has symptoms including feeding disorder/dysphagia/swallowing/sucking disorder/esophageal dyskinesia, early death/lethality and hepatomegaly/liver enlargement (excluding storage disease). An important gene associated with Gamma Heavy Chain Disease is IGHG3 (immunoglobulin heavy constant gamma 3 (G3m marker)). Affiliated tissues include lymph node, thyroid and t cells.

Disease Ontology:8 A heavy chain disease that results from an overproduction of gamma antibody (igg).

Aliases & Classifications for Gamma Heavy Chain Disease

Sources:
8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 49Orphanet, 61UMLS, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
franklin disease:
Prevalence: <1/1000000; Age of onset: Adulthood; Age of death: Any age


Aliases & Descriptions:

gamma heavy chain disease 8 43 10
franklin disease 43 49
gamma heavy chain deposition disease 43
gamma heavy-chain disease 49
igg heavy chain disease 43
gamma-chain disease 61
franklin's disease 8
gamma-hcd 49


External Ids:

Disease Ontology8 DOID:0060127
ICD10 via Orphanet26 C88.2
SNOMED-CT via Orphanet58 109984001

Related Diseases for Gamma Heavy Chain Disease

Sources:
17GeneCards, 18GeneDecks
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Diseases in the Gamma Heavy Chain Disease family:

heavy chain disease alpha heavy-chain disease

Diseases related to Gamma Heavy Chain Disease via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show all 13)
idRelated DiseaseScoreTop Affiliating Genes
1heavy chain deposition disease10.6
2splenomegaly10.4
3amyloidosis10.3
4light chain deposition disease10.3
5thyroid lymphoma10.2
6pancytopenia10.2
7alpha chain disease10.2
8mu chain disease10.2
9lymphoplasmacytic lymphoma10.2
10splenic marginal zone lymphoma10.2
11splenic disease10.2
12alpha heavy-chain disease10.2
13progressive multifocal leukoencephalopathy10.1

Graphical network of diseases related to Gamma Heavy Chain Disease:



Diseases related to gamma heavy chain disease

Clinical Features for Gamma Heavy Chain Disease

Sources:
49Orphanet
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Symptoms:

49 (show all 21)
  • feeding disorder/dysphagia/swallowing/sucking disorder/esophageal dyskinesia
  • early death/lethality
  • hepatomegaly/liver enlargement (excluding storage disease)
  • splenomegaly
  • repeat respiratory infections
  • asthenia/fatigue/weakness
  • fever/chilling
  • anaemia
  • cutaneous rash
  • peripheral neuropathy
  • anomalies of tongue, gingiva and oral mucosa
  • autoimmunity/autoimmune reaction/autoantibodies
  • osteolysis/osteoclasia/bone destruction/erosions
  • thrombocytopenia/thrombopenia
  • hemolytic anemia
  • purpura/petichiae
  • lymphadenopathy/polyadenopathies
  • palate anomalies
  • monoclonal immunoglobulins/gammapathy/dysglobulinemia
  • lymphocytes anomalies
  • bone marrow/medullar infiltration

Drugs & Therapeutics for Gamma Heavy Chain Disease

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

Approved drugs:

Search CenterWatch for Gamma Heavy Chain Disease

Drug clinical trials:

Search ClinicalTrials for Gamma Heavy Chain Disease

Search NIH Clinical Center for Gamma Heavy Chain Disease

Search CenterWatch for Gamma Heavy Chain Disease

Genetic Tests for Gamma Heavy Chain Disease

Anatomical Context for Gamma Heavy Chain Disease

Sources:
33MalaCards
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MalaCards organs/tissues related to Gamma Heavy Chain Disease:

33
Lymph node, Thyroid, T cells

Animal Models for Gamma Heavy Chain Disease or affiliated genes

Sources:
28inGenious Targeting Laboratory
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Publications for Gamma Heavy Chain Disease

Sources:
51PubMed
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Articles related to Gamma Heavy Chain Disease:

(show top 50)    (show all 91)
idTitleAuthorsYear
1
Unusual concurrence of T-cell large granular lymphocytic leukemia with Franklin disease (gamma heavy chain disease) manifested with massive splenomegaly. (22694793)
2013
2
Gamma heavy-chain disease: defining the spectrum of associated lymphoproliferative disorders through analysis of 13 cases. (22301495)
2012
3
Gamma heavy chain disease in a patient with rheumatoid arthritis--a laboratory evaluation. (23092068)
2012
4
Gamma heavy chain disease in a patient with diabetes and chronic renal insufficiency: diagnostic assessment of the heavy chain fragment. (18348312)
2008
5
Clinical remission and histopathological resolution of nodular lesions in a patient with gamma3 heavy-chain deposition disease. (18538103)
2008
6
Is there a pathogenic link between gamma heavy chain disease and chronic arthritis? (10647957)
2000
7
Electrophoretic characterization of a gamma-1-heavy chain disease. (10985478)
2000
8
The amino acid sequence of a monoclonal gamma 3-heavy chain from a patient with articular gamma-heavy chain deposition disease. (10849371)
2000
9
Gamma-heavy chain deposition disease showing nodular glomerulosclerosis. (8719552)
1995
10
Biological diagnosis of a gamma-1-heavy chain disease in an asymptomatic patient. (7720844)
1995
11
Primary SjAPgren's syndrome and gamma heavy chain disease. (7991295)
1994
12
First report of fludarabine in gamma-heavy chain disease. (7819086)
1994
13
Atrophie blanche in a patient with gamma-heavy-chain disease. (1899331)
1991
14
Cutaneous manifestations associated with gamma heavy chain disease. Report of an unusual case and review of literature. (2121807)
1990
15
Gamma heavy chain disease and rheumatoid arthritis. (2658067)
1989
16
Gamma heavy chain &quot;disease&quot;: heterogeneity of the clinicopathologic features. Report of 16 cases and review of the literature. (2509855)
1989
17
Cutaneous lesions in gamma heavy-chain disease. (3138950)
1988
18
Multiple osteolytic lesions in a case of gamma heavy chain disease: roentgenographic findings. (3133921)
1988
19
Gamma-chain heavy-chain disease with primary thyroid plasmacytoma. (3094476)
1986
20
Gamma heavy chain disease DUB: clinical, immunochemical and pathological studies. (3932151)
1985
21
Gamma heavy chain disease: report of a case associated with trisomy of chromosome 7. (3917846)
1985
22
Gamma heavy chain disease. Where the heavy chains arise. (6404156)
1983
23
Gamma heavy chain disease. Ultrastructural and immunohistochemical study. (6414241)
1983
24
Biochemical and biosynthetic studies of a crystallizable human gamma 1 heavy-chain disease protein. (6410498)
1983
25
Gamma heavy chain disease in man: cDNA sequence supports partial gene deletion model. (6808505)
1982
26
Gamma heavy chain disease and giant lymph node hyperplasia in a patient with impaired T cell function. (6811785)
1982
27
A new case of gamma-heavy chain disease. Clinical and immunochemical studies. (6806895)
1982
28
The histopathology of gamma heavy-chain disease. (6814232)
1982
29
The pathology of gamma heavy chain disease: report of a case with morphologic progression from lymphocytic to plasmacytic proliferation. (6790158)
1981
30
Diagnosis of gamma heavy-chain disease. (6792974)
1981
31
Primary structure of human gamma 3 immunoglobulin deletion mutant: gamma 3 heavy-chain disease protein Wis. (6774747)
1980
32
A gamma l heavy-chain disease protein *EST) lacking the entire VH and CHl domains. (6777865)
1980
33
A new case of gamma heavy chain disease: clinical, immunochemical and structural characterization. (91266)
1979
34
Gamma 3-heavy-chain disease (gamma 3-HCD) in a young patient with Down syndrome. Study of peripheral blood lymphocytes and of susceptibility to infection. (156611)
1979
35
An unusual case of a plasma cell neoplasm with an IgG3lambda myeloma and a gamma3 heavy chain disease protein. (412532)
1978
36
Gamma heavy chain disease in man: synthesis of a deleted gamma3 immunoglobulin by lymphoid cells in short and long term tissue culture. (80297)
1978
37
Pathological studies on a long-term survived case of gamma heavy chain disease--a brief review of 30 reported cases and a proposal for histological typing. (104544)
1978
38
Monoclonal IgM cryoglobulinemia associated with gamma-3 heavy chain disease: immunochemical and biochemical studies. (97087)
1978
39
The structural heterogeneity among degraded gamma heavy chain disease proteins. (102697)
1978
40
Gamma heavy chain disease in man: translation and partial purification of mRNA coding for the deleted protein. (105356)
1978
41
Gamma heavy chain disease; report of an autopsy case with a review of the literatures. (822689)
1976
42
Gamma heavy chain disease in a young girl. (828123)
1976
43
A case of gamma heavy chain disease associated with autoimmune haemolytic anaemia: clinical, haematological, immunological and pathological details. (821137)
1976
44
A new immunoglobulin variant: gamma3 heavy chain disease protein CHI. (818639)
1976
45
Gamma heavy chain disease: rapid, sustained response to cyclophosphamide and prednisone. (1131420)
1975
46
In vitro production of an amyloid-like substance from gamma 3 heavy chain disease protein. (4609897)
1974
47
Gamma heavy chain disease. Reports of three patients. (4562896)
1972
48
Gamma-heavy chain disease. Preliminary studies in three cases. (4137680)
1972
49
Heavy chain disease of the gamma (gamma M) type: report of the first case. (4193528)
1970
50
HEAVY CHAIN DISEASE- A NEW DISORDER OF SERUM GAMMA-GLOBULINS : REPORT OF THE FIRST CASE. (14209281)
1964

Genetic Variations for Gamma Heavy Chain Disease

Expression for genes affiliated with Gamma Heavy Chain Disease

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Gamma Heavy Chain Disease

Search GEO for disease gene expression data for Gamma Heavy Chain Disease.

Pathways for genes affiliated with Gamma Heavy Chain Disease

Compounds for genes affiliated with Gamma Heavy Chain Disease

GO Terms for genes affiliated with Gamma Heavy Chain Disease

Products for genes affiliated with Gamma Heavy Chain Disease

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Gamma Heavy Chain Disease

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