TEMPI Syndrome

Preferred Label : TEMPI Syndrome;

NCIt definition : A rare syndrome characterized by telangiectasias, elevated erythropoietin level and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting. It is best classified as a type of plasma cell dyscrasia with paraneoplastic manifestations.;

Neoplastic status : Malignant;

Details

Origin ID

C121656;

UMLS CUI

C3854394;

Currated CISMeF NLP mapping
- TEMPI syndrome [ORDO Disease]
- TEMPI syndrome [MedDRA Preferred Term]
- Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder) [SNOMED CT concept]
Disease excludes abnormal cell
- Neoplastic T-Lymphocyte and Neoplastic Natural Killer Cell [NCIt concept]
Disease excludes normal cell origin
- Myeloid Cell [NCIt concept]
- Precursor B-Lymphoblast [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- TEMPI syndrome [ORDO Disease]
- TEMPI syndrome [MedDRA Preferred Term]
- Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder) [SNOMED CT concept]
disease_has_abnormal_cell
- Abnormal Lymphocyte [NCIt concept]
- Neoplastic B-Lymphocyte [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Hematopoietic and Lymphoid Cell [NCIt concept]
- Neoplastic Lymphocyte [NCIt concept]
- Neoplastic Plasma Cell [NCIt concept]
disease_has_associated_disease
- Paraneoplastic Syndrome [NCIt concept]
- Telangiectasia [NCIt concept]
disease_has_finding
- Elevated Erythropoietin [NCIt concept]
- Erythrocytosis [NCIt concept]
- Intrapulmonary Shunt [NCIt concept]
- Monoclonal Gammopathy [NCIt concept]
- Perinephric Fluid Collection [NCIt concept]
disease_has_normal_cell_origin
- B-Lymphocyte [NCIt concept]
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Lymphocyte [NCIt concept]
- Mature B-Lymphocyte [NCIt concept]
- Plasma Cell [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic and Lymphoid Tissue [NCIt concept]
- Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Hematopoietic and Lymphatic System [NCIt concept]
- Lymphatic System [NCIt concept]

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