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 peripheral neuropathy, osteolysis/osteoclasia/bone destruction/erosions and autoimmunity/autoimmune reaction/autoantibodies. An important gene associated with Gamma Heavy Chain Disease is IGHG3 (immunoglobulin heavy constant gamma 3 (G3m marker)). Affiliated tissues include bone, bone marrow and tongue.

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

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, Bone marrow, Tongue, Liver, Thyroid, T cells, 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 in a patient with rheumatoid arthritis--a laboratory evaluation. (23092068)
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
Unusual gamma heavy chain disease protein in a patient with splenic marginal-zone lymphoma. (16536920)
2006
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 screening showing a discrepancy between electrophoretic and nephelometric determinations of serum gamma globulin concentration. (12227865)
2002
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
Chronic arthritis and gamma heavy chain disease: coincidence or pathogenic link? (9751465)
1998
11
Gamma-heavy chain deposition disease showing nodular glomerulosclerosis. (8719552)
1995
12
Free fragments of gamma chain in the urine. A possible source of confusion with gamma heavy-chain disease. (8160637)
1994
13
Primary SjAPgren's syndrome and gamma heavy chain disease. (7991295)
1994
14
First report of fludarabine in gamma-heavy chain disease. (7819086)
1994
15
Unusual sequence of immunoglobulin L-chain rearrangements in a gamma heavy chain disease patient. (1519038)
1992
16
Gamma heavy chain disease associated with Hodgkin's disease. Clinical, pathologic, and immunologic features of one case. (2495168)
1989
17
Gamma heavy chain disease in man. Genomic sequence reveals two noncontiguous deletions in a single gene. (3139711)
1988
18
Rapid evolution in the immunochemical findings of a gamma heavy chain disease. (3124986)
1988
19
Multiple osteolytic lesions in a case of gamma heavy chain disease: roentgenographic findings. (3133921)
1988
20
Gamma heavy chain disease DUB: clinical, immunochemical and pathological studies. (3932151)
1985
21
Gamma 3 heavy chain disease and monoclonal IgMk in a patient with a gastrointestinal lymphoma. (3924773)
1985
22
Gamma heavy chain disease: report of a case associated with trisomy of chromosome 7. (3917846)
1985
23
Gamma heavy chain disease studied by two-dimensional electrophoresis and immuno-blotting techniques. (6437695)
1984
24
Gamma heavy chain disease. Ultrastructural and immunohistochemical study. (6414241)
1983
25
Gamma heavy chain disease in man: cDNA sequence supports partial gene deletion model. (6808505)
1982
26
A new case of gamma-heavy chain disease. Clinical and immunochemical studies. (6806895)
1982
27
The diverse picture of gamma heavy-chain disease. Report of seven cases and review of literature. (6789012)
1981
28
The pathology of gamma heavy chain disease: report of a case with morphologic progression from lymphocytic to plasmacytic proliferation. (6790158)
1981
29
Diagnosis of gamma heavy-chain disease. (6792974)
1981
30
Gamma heavy chain disease protein CHA: immunological and structural studies. (6796821)
1981
31
Gamma heavy chain disease with an unusually benign course. (6769634)
1980
32
Primary structure of human gamma 3 immunoglobulin deletion mutant: gamma 3 heavy-chain disease protein Wis. (6774747)
1980
33
A gamma l heavy-chain disease protein *EST) lacking the entire VH and CHl domains. (6777865)
1980
34
A case of gamma 3 heavy chain disease with vacuolated plasma cells: a clinical, immunological, and ultrastructural study. (109474)
1979
35
A new case of gamma heavy chain disease: clinical, immunochemical and structural characterization. (91266)
1979
36
Gamma heavy chain disease seen initially as gastric neoplasm. (96746)
1978
37
A new case of gamma-heavy chain disease. (417549)
1978
38
Physicochemical and immunochemical properties of gamma l heavy chain disease protein BAZ. (100413)
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: clinical aspects and characterization of a deleted, noncovalently linked gamma1 heavy chain dimer (BAZ). (402960)
1977
42
Gamma heavy chain disease--presenting as pancytopenia and splenomegaly. (403613)
1977
43
Gamma heavy chain disease; report of an autopsy case with a review of the literatures. (822689)
1976
44
Gamma heavy chain disease in a young girl. (828123)
1976
45
N- and C-terminal amino acid sequences of a gamma-heavy chain disease protein YOK. (819357)
1976
46
A case of gamma heavy chain disease associated with autoimmune haemolytic anaemia: clinical, haematological, immunological and pathological details. (821137)
1976
47
Clinical findings in a case of newly defined gamma heavy chain disease protein. (4747582)
1973
48
Gamma-heavy chain disease. Preliminary studies in three cases. (4137680)
1972
49
A new type of human gamma heavy chain disease protein: immunochemical and physical characteristics. (4194087)
1970
50
Heavy chain disease of the gamma (gamma M) type: report of the first case. (4193528)
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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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Gamma Heavy Chain Disease

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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