MCID: GMM003
MIFTS: 35

Gamma Heavy Chain Disease malady

Blood diseases, Cancer diseases categories

Summaries for Gamma Heavy Chain Disease

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8Disease Ontology, 42NIH Rare Diseases, 32MalaCards
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NIH Rare Diseases:42 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 rheumatoid arthritis, and has symptoms including purpura/petichiae, lymphadenopathy/polyadenopathies and palate anomalies. An important gene associated with Gamma Heavy Chain Disease is IGHG3 (immunoglobulin heavy constant gamma 3 (G3m marker)). Affiliated tissues include bone, liver and bone marrow.

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

Aliases & Classifications for Gamma Heavy Chain Disease

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 48Orphanet, 60UMLS, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet
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Classifications:

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


Characteristics (Orphanet epidemiological data):

48
franklin disease:
Prevalence: <1/1000000; Age of onset: Adulthood; Age of death: Any age


Aliases & Descriptions:

gamma heavy chain disease 8 42 10
franklin disease 42 48
gamma heavy chain deposition disease 42
gamma heavy-chain disease 48
igg heavy chain disease 42
gamma-chain disease 60
franklin's disease 8
gamma-hcd 48


External Ids:

Disease Ontology8 DOID:0060127
ICD10 via Orphanet26 C88.2
SNOMED-CT via Orphanet57 109984001

Related Diseases for Gamma Heavy Chain Disease

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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 15)
idRelated DiseaseScoreTop Affiliating Genes
1heavy chain disease11.1
2rheumatoid arthritis10.4
3amyloidosis10.3
4thyroid lymphoma10.2
5pancytopenia10.2
6alpha chain disease10.2
7lymphoplasmacytic lymphoma10.2
8t-cell large granular lymphocyte leukemia10.2
9splenic marginal zone lymphoma10.2
10chromosomal disease10.2
11leukemia10.2
12splenic disease10.2
13t-cell leukemia10.2
14thyroiditis10.2
15progressive multifocal leukoencephalopathy10.0

Graphical network of diseases related to Gamma Heavy Chain Disease:



Diseases related to gamma heavy chain disease

Clinical Features for Gamma Heavy Chain Disease

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48Orphanet
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Symptoms:

48 (show all 21)
  • purpura/petichiae
  • lymphadenopathy/polyadenopathies
  • palate anomalies
  • monoclonal immunoglobulins/gammapathy/dysglobulinemia
  • lymphocytes anomalies
  • bone marrow/medullar infiltration
  • 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

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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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

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Anatomical Context for Gamma Heavy Chain Disease

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Sources:
32MalaCards
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MalaCards organs/tissues related to Gamma Heavy Chain Disease:

32
Bone, Liver, Bone marrow, Tongue, T cells, Thyroid, Lymph node

Animal Models for Gamma Heavy Chain Disease or affiliated genes

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Publications for Gamma Heavy Chain Disease

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50PubMed
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Articles related to Gamma Heavy Chain Disease:

(show top 50)    (show all 76)
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 underlying lymphoplasmacytic lymphoma of the thyroid. Report of a case and comparison with other reported cases with thyroid involvement. (22561184)
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
Combination of rituximab and chemotherapy showing anti-tumor effect in gamma heavy chain disease expressing CD20. (18839436)
2008
6
Development of systemic lambda-light chain amyloidosis in a patient with gamma-heavy chain deposition disease during long-term follow-up. (15673693)
2005
7
Gamma-heavy chain disease: review of 23 cases. (12861101)
2003
8
Composite nodular lymphocyte-predominance Hodgkin disease and gamma-heavy-chain disease: a case report and review of the literature. (11371236)
2001
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
The amino acid sequence of a monoclonal gamma 3-heavy chain from a patient with articular gamma-heavy chain deposition disease. (10849371)
2000
11
Gamma-heavy chain deposition disease showing nodular glomerulosclerosis. (8719552)
1995
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
Atrophie blanche in a patient with gamma-heavy-chain disease. (1899331)
1991
16
Cutaneous manifestations associated with gamma heavy chain disease. Report of an unusual case and review of literature. (2121807)
1990
17
Gamma heavy chain disease and rheumatoid arthritis. (2658067)
1989
18
Gamma heavy chain disease associated with Hodgkin's disease. Clinical, pathologic, and immunologic features of one case. (2495168)
1989
19
Gamma heavy chain disease in man. Genomic sequence reveals two noncontiguous deletions in a single gene. (3139711)
1988
20
Multiple osteolytic lesions in a case of gamma heavy chain disease: roentgenographic findings. (3133921)
1988
21
Osteolytic gamma heavy chain disease. (3115824)
1987
22
Gamma heavy-chain disease involving upper airway. (3932940)
1985
23
Gamma 3 heavy chain disease and monoclonal IgMk in a patient with a gastrointestinal lymphoma. (3924773)
1985
24
Gamma heavy chain disease: report of a case associated with trisomy of chromosome 7. (3917846)
1985
25
Gamma heavy chain disease. Ultrastructural and immunohistochemical study. (6414241)
1983
26
Biochemical and biosynthetic studies of a crystallizable human gamma 1 heavy-chain disease protein. (6410498)
1983
27
Gamma heavy chain disease and giant lymph node hyperplasia in a patient with impaired T cell function. (6811785)
1982
28
A new case of gamma-heavy chain disease. Clinical and immunochemical studies. (6806895)
1982
29
The histopathology of gamma heavy-chain disease. (6814232)
1982
30
The diverse picture of gamma heavy-chain disease. Report of seven cases and review of literature. (6789012)
1981
31
Gamma heavy chain disease with an unusually benign course. (6769634)
1980
32
A gamma l heavy-chain disease protein *EST) lacking the entire VH and CHl domains. (6777865)
1980
33
A case of gamma 3 heavy chain disease with vacuolated plasma cells: a clinical, immunological, and ultrastructural study. (109474)
1979
34
A new case of gamma heavy chain disease: clinical, immunochemical and structural characterization. (91266)
1979
35
Gamma heavy chain disease in man: synthesis of a deleted gamma3 immunoglobulin by lymphoid cells in short and long term tissue culture. (80297)
1978
36
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
37
Gamma heavy chain disease seen initially as gastric neoplasm. (96746)
1978
38
A new case of gamma-heavy chain disease. (417549)
1978
39
Physicochemical and immunochemical properties of gamma l heavy chain disease protein BAZ. (100413)
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--presenting as pancytopenia and splenomegaly. (403613)
1977
45
Gamma heavy chain disease; report of an autopsy case with a review of the literatures. (822689)
1976
46
Gamma heavy chain disease in a young girl. (828123)
1976
47
N- and C-terminal amino acid sequences of a gamma-heavy chain disease protein YOK. (819357)
1976
48
Clinical findings in a case of newly defined gamma heavy chain disease protein. (4747582)
1973
49
Gamma-heavy chain disease. Preliminary studies in three cases. (4137680)
1972
50
A new type of human gamma heavy chain disease protein: immunochemical and physical characteristics. (4194087)
1970

Genetic Variations for Gamma Heavy Chain Disease

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Expression for genes affiliated with Gamma Heavy Chain Disease

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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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

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Compounds for genes affiliated with Gamma Heavy Chain Disease

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GO Terms for genes affiliated with Gamma Heavy Chain Disease

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Products for genes affiliated with Gamma Heavy Chain Disease

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Gamma Heavy Chain Disease

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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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet