Pregerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma

Definition

A recently recognized variant of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) that lacks somatic hypermutations of the Immunoglobulin heavy chain (IGH) genes, implying pregerminal center B-cell origin. Microarray gene expression profiling studies have demonstrated the expression of ZAP-70 gene (Syk family tyrosine kinase) in this subset of CLL/CLL. Patients with this variant of CLL/SLL have an unfavorable prognosis compared to those with somatic hypermutations of the IGH genes, with a median survival of approximately 6-8 years.

Also known as chronic lymphocytic leukemia/small lymphocytic lymphoma with unmutated Immunoglobulin heavy chain variable-region Gene, CLL/SLL with unmutated IGVH, pregerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma — per MONDO

Also identified as

• DOID 8144 per MONDO
• NCIT C37204 per MONDO
• UMLS C1333038 per MONDO

Affected Organs