Polyclonal hyperviscosity syndrome
- Type
- Clinical syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, All ages, Elderly
- Status
- active
A rare hematologic disease characterized by high serum viscosity due to polyclonal expansion of immunoglobulins, most commonly in the context of Waldenström's macroglobulinemia, 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 hemorrhage, headache, stroke, pulmonary hypertension, and congestive heart failure.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Polyclonal hyperviscosity syndrome | |
| fr | Syndrome d'hyperviscosité polyclonale | |
| de | Polyklonales Hyperviskositätssyndrom | |
| es | Síndrome de hiperviscosidad policlonal | |
| it | Sindrome da iperviscosità policlonale | |
| nl | Polyklonale hyperviscositeitssyndroom | |
| pt | Síndrome de hiperviscosidade policlonal | |
| pl | Zespół nadlepkości poliklonalnej | |
| cs | Polyklonální hyperviskózní syndrom | |
| uk | Синдром поліклональної гіпервязкості | |
| zh | 多克隆高粘度综合征 | |
| tr | Poliklonal hiperviskozite sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D89.0 | NTBT | attributed | validated |
| MONDO | 0018678 | E | validated | |
| UMLS | C5681102 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:97992Rare hematologic disease
rare hematological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/450322
- This record as JSON: /data/d/450322.json