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Polyclonal hyperviscosity syndrome

MedGen UID:
1814466
Concept ID:
C5681102
Disease or Syndrome
Synonym: polyclonal hyperviscosity syndrome
SNOMED CT: Polyclonal hyperviscosity syndrome (1251448003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018678
Orphanet: ORPHA450322

Definition

A rare haematologic disease characterised by high serum viscosity due to polyclonal expansion of immunoglobulins, most commonly in the context of Waldenström macroglobulinaemia, as well as a variety of disorders of immune dysregulation. Patients present with signs and symptoms involving multiple organs, such as bleeding diathesis, mucosal bleeding, retinal haemorrhage, headache, stroke, pulmonary hypertension and congestive heart failure. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPolyclonal hyperviscosity syndrome

Professional guidelines

PubMed

Zhao EJ, Cheng CV, Mattman A, Chen LYC
Lancet Haematol 2021 May;8(5):e365-e375. doi: 10.1016/S2352-3026(21)00056-9. PMID: 33894171

Recent clinical studies

Prognosis

Hadler NM, Gabriel D, Su Chung K, Teague P, Napier MA
Arthritis Rheum 1977 Sep-Oct;20(7):1388-95. doi: 10.1002/art.1780200713. PMID: 911356

Supplemental Content

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