National Cancer Institute Thesaurus

Last uploaded: February 23, 2024
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Preferred Name

Richter Syndrome
Synonyms

Richter transformation

Richter syndrome

Richter Syndrome

Richter's Syndrome

Richter's Transformation
Definitions

Transformation of chronic lymphocytic leukemia into aggressive non-Hodgkin lymphoma, usually diffuse large B-cell lymphoma (immunoblastic or centroblastic variant). Occasional cases of transformation to Hodgkin lymphoma have also been described, particularly in patients treated with purine nucleotide analogues. Molecular genetic studies suggest that in approximately half of the cases, the lymphoma is clonally related to the underlying chronic lymphocytic leukemia, whereas in the remaining cases the lymphoma probably represents a secondary, unrelated neoplasm.
ID

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C35424
ALT_DEFINITION

A rare condition in which chronic lymphocytic leukemia (CLL) changes into a fast-growing type of lymphoma. Symptoms of Richter syndrome include fever, loss of weight and muscle mass, and other health problems.
code

C35424
Concept_In_Subset

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C116977

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C118168
Contributing_Source

CTRP
DEFINITION

Transformation of chronic lymphocytic leukemia into aggressive non-Hodgkin lymphoma, usually diffuse large B-cell lymphoma (immunoblastic or centroblastic variant). Occasional cases of transformation to Hodgkin lymphoma have also been described, particularly in patients treated with purine nucleotide analogues. Molecular genetic studies suggest that in approximately half of the cases, the lymphoma is clonally related to the underlying chronic lymphocytic leukemia, whereas in the remaining cases the lymphoma probably represents a secondary, unrelated neoplasm.
Display_Name

Richter Syndrome
FULL_SYN

Richter transformation

Richter syndrome

Richter Syndrome

Richter's Syndrome

Richter's Transformation
label

Richter Syndrome
Legacy Concept Name

Richter_s_Syndrome
Preferred_Name

Richter Syndrome
prefixIRI

Thesaurus:C35424
Semantic_Type

Disease or Syndrome
UMLS_CUI

C0349631
subClassOf

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C54705
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