MCID: GMM003
MIFTS: 37

Gamma Heavy Chain Disease malady

Rare diseases, Blood diseases, Cancer diseases, Immune diseases, Gastrointestinal diseases categories

Summaries for Gamma Heavy Chain Disease

About this section
Sources:
9Disease Ontology, 44NIH Rare Diseases, 34MalaCards
See all sources

Fully expand this MalaCard

Download this MalaCard
NIH Rare Diseases:44 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 disease and heavy chain deposition disease, and has symptoms including purpura/petichiae, lymphadenopathy/polyadenopathies and bone marrow/medullar infiltration. An important gene associated with Gamma Heavy Chain Disease is IGHG3 (immunoglobulin heavy constant gamma 3 (G3m marker)). Affiliated tissues include bone, tongue and liver.

Disease Ontology:9 A heavy chain disease that results from an overproduction of gamma antibody (igg).

Aliases & Classifications for Gamma Heavy Chain Disease

About this section
Sources:
9Disease Ontology, 44NIH Rare Diseases, 11DISEASES, 50Orphanet, 63UMLS, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet
See all sources

Classifications:



Characteristics (Orphanet epidemiological data):

50
franklin disease:
Prevalence: <1/1000000; Age of onset: Adulthood; Age of death: Any age


Aliases & Descriptions:

gamma heavy chain disease 9 44 11
franklin disease 44 50
gamma heavy chain deposition disease 44
gamma heavy-chain disease 50
igg heavy chain disease 44
gamma-chain disease 63
franklin's disease 9
gamma-hcd 50


External Ids:

Disease Ontology9 DOID:0060127
ICD10 via Orphanet27 C88.2
SNOMED-CT via Orphanet60 109984001

Related Diseases for Gamma Heavy Chain Disease

About this section
Sources:
18GeneCards, 19GeneDecks
See all sources

Graphical network of diseases related to Gamma Heavy Chain Disease:



Diseases related to gamma heavy chain disease

Symptoms for Gamma Heavy Chain Disease

About this section
Sources:
50Orphanet
See all sources


Symptoms:

50 (show all 21)
  • purpura/petichiae
  • lymphadenopathy/polyadenopathies
  • bone marrow/medullar infiltration
  • palate anomalies
  • monoclonal immunoglobulins/gammapathy/dysglobulinemia
  • lymphocytes anomalies
  • hemolytic anemia
  • thrombocytopenia/thrombopenia
  • 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
  • feeding disorder/dysphagia/swallowing/sucking disorder/esophageal dyskinesia

Drugs & Therapeutics for Gamma Heavy Chain Disease

About this section
Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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

About this section

Anatomical Context for Gamma Heavy Chain Disease

About this section
Sources:
34MalaCards
See all sources

MalaCards organs/tissues related to Gamma Heavy Chain Disease:

34
Bone, Tongue, Liver, Bone marrow, T cells, Lymph node, Thyroid

Animal Models for Gamma Heavy Chain Disease or affiliated genes

About this section

Publications for Gamma Heavy Chain Disease

About this section
Sources:
53PubMed
See all sources

Articles related to Gamma Heavy Chain Disease:

(show top 50)    (show all 62)
idTitleAuthorsYear
1
Gamma heavy-chain disease: defining the spectrum of associated lymphoproliferative disorders through analysis of 13 cases. (22301495)
2012
2
Gamma heavy chain disease in a patient with rheumatoid arthritis--a laboratory evaluation. (23092068)
2012
3
Gamma heavy chain disease in a patient with diabetes and chronic renal insufficiency: diagnostic assessment of the heavy chain fragment. (18348312)
2008
4
Combination of rituximab and chemotherapy showing anti-tumor effect in gamma heavy chain disease expressing CD20. (18839436)
2008
5
Unusual gamma heavy chain disease protein in a patient with splenic marginal-zone lymphoma. (16536920)
2006
6
Gamma-heavy chain disease: review of 23 cases. (12861101)
2003
7
Composite nodular lymphocyte-predominance Hodgkin disease and gamma-heavy-chain disease: a case report and review of the literature. (11371236)
2001
8
Is there a pathogenic link between gamma heavy chain disease and chronic arthritis? (10647957)
2000
9
Gamma heavy chain disease in man: independent structural abnormalities and reduced transcription of a functionally rearranged lambda L-chain gene result in the absence of L-chains. (10950937)
2000
10
Chronic arthritis and gamma heavy chain disease: coincidence or pathogenic link? (9751465)
1998
11
Free fragments of gamma chain in the urine. A possible source of confusion with gamma heavy-chain disease. (8160637)
1994
12
Primary SjAPgren's syndrome and gamma heavy chain disease. (7991295)
1994
13
First report of fludarabine in gamma-heavy chain disease. (7819086)
1994
14
Unusual sequence of immunoglobulin L-chain rearrangements in a gamma heavy chain disease patient. (1519038)
1992
15
N-ras oncogene activation in a patient with gamma heavy chain disease. (1288299)
1992
16
Atrophie blanche in a patient with gamma-heavy-chain disease. (1899331)
1991
17
Cutaneous manifestations associated with gamma heavy chain disease. Report of an unusual case and review of literature. (2121807)
1990
18
Gamma heavy chain disease and rheumatoid arthritis. (2658067)
1989
19
Gamma heavy chain disease associated with Hodgkin's disease. Clinical, pathologic, and immunologic features of one case. (2495168)
1989
20
Gamma heavy chain disease in man. Genomic sequence reveals two noncontiguous deletions in a single gene. (3139711)
1988
21
Rapid evolution in the immunochemical findings of a gamma heavy chain disease. (3124986)
1988
22
Osteolytic gamma heavy chain disease. (3115824)
1987
23
Gamma heavy chain disease DUB: clinical, immunochemical and pathological studies. (3932151)
1985
24
Gamma heavy-chain disease involving upper airway. (3932940)
1985
25
Gamma heavy chain disease: report of a case associated with trisomy of chromosome 7. (3917846)
1985
26
Gamma heavy chain disease. Where the heavy chains arise. (6404156)
1983
27
Gamma heavy chain disease. Ultrastructural and immunohistochemical study. (6414241)
1983
28
Gamma heavy chain disease in man: cDNA sequence supports partial gene deletion model. (6808505)
1982
29
Gamma heavy chain disease and giant lymph node hyperplasia in a patient with impaired T cell function. (6811785)
1982
30
A new case of gamma-heavy chain disease. Clinical and immunochemical studies. (6806895)
1982
31
The diverse picture of gamma heavy-chain disease. Report of seven cases and review of literature. (6789012)
1981
32
The pathology of gamma heavy chain disease: report of a case with morphologic progression from lymphocytic to plasmacytic proliferation. (6790158)
1981
33
Diagnosis of gamma heavy-chain disease. (6792974)
1981
34
Gamma heavy chain disease with an unusually benign course. (6769634)
1980
35
A new case of gamma heavy chain disease: clinical, immunochemical and structural characterization. (91266)
1979
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
Gamma heavy chain disease seen initially as gastric neoplasm. (96746)
1978
39
A new case of gamma-heavy chain disease. (417549)
1978
40
The structural heterogeneity among degraded gamma heavy chain disease proteins. (102697)
1978
41
Gamma heavy chain disease simulating alpha chain disease. (105971)
1978
42
Gamma heavy chain disease in man: translation and partial purification of mRNA coding for the deleted protein. (105356)
1978
43
Gamma heavy chain disease: clinical aspects and characterization of a deleted, noncovalently linked gamma1 heavy chain dimer (BAZ). (402960)
1977
44
Gamma heavy chain disease; report of an autopsy case with a review of the literatures. (822689)
1976
45
Gamma heavy chain disease in a young girl. (828123)
1976
46
N- and C-terminal amino acid sequences of a gamma-heavy chain disease protein YOK. (819357)
1976
47
A case of gamma heavy chain disease associated with autoimmune haemolytic anaemia: clinical, haematological, immunological and pathological details. (821137)
1976
48
Gamma heavy chain disease: rapid, sustained response to cyclophosphamide and prednisone. (1131420)
1975
49
Gamma heavy chain disease. Reports of three patients. (4562896)
1972
50
A new type of human gamma heavy chain disease protein: immunochemical and physical characteristics. (4194087)
1970

Variations for Gamma Heavy Chain Disease

About this section

Expression for genes affiliated with Gamma Heavy Chain Disease

About this section
Sources:
2BioGPS, 16Gene 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

About this section

Compounds for genes affiliated with Gamma Heavy Chain Disease

About this section

GO Terms for genes affiliated with Gamma Heavy Chain Disease

About this section

Products for genes affiliated with Gamma Heavy Chain Disease

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Gamma Heavy Chain Disease

About this section
4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet