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Gamma heavy chain disease (clinical)

MedGen UID:
42374
Concept ID:
C0018854
Disease or Syndrome
Synonyms: Franklin disease; Gamma heavy chain deposition disease; Gamma heavy chain disease; gamma-Chain Disease; IgG heavy chain disease
SNOMED CT: Gamma heavy chain disease (clinical) (109984001); Gamma heavy chain disease (6381009); Franklin disease (6381009); Gamma heavy chain disease (109984001)
 
Monarch Initiative: MONDO:0015046
Orphanet: ORPHA100026

Definition

A type of HCD characterized by the production of incomplete monoclonal gamma-heavy chains without associated light chains. The clinical presentation most commonly resembles that of patients with systemic lymphoproliferative/autoimmune diseases. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGamma heavy chain disease (clinical)

Professional guidelines

PubMed

Hampel PJ, Parikh SA
Blood Cancer J 2022 Nov 29;12(11):161. doi: 10.1038/s41408-022-00756-9. PMID: 36446777Free PMC Article
Gertz MA
Am J Hematol 2022 Jun 1;97(6):818-829. Epub 2022 Apr 25 doi: 10.1002/ajh.26569. PMID: 35429180
Jain P, Wang M
Am J Hematol 2019 Jun;94(6):710-725. Epub 2019 Apr 19 doi: 10.1002/ajh.25487. PMID: 30963600

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

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