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Chronic Lymphocytic Leukemia (CLL) with Borderline Immunoglobulin Heavy Chain Mutational Status, a Rare Subgroup of CLL with Variable Disease Course

Title: Chronic Lymphocytic Leukemia (CLL) with Borderline Immunoglobulin Heavy Chain Mutational Status, a Rare Subgroup of CLL with Variable Disease Course
Authors: Angotzi, Francesco; Cellini, Alessandro; Ruocco, Valeria; Cavarretta, Chiara Adele; Zatta, Ivan; Serafin, Andrea; Pravato, Stefano; Pagnin, Elisa; Bonaldi, Laura; Frezzato, Federica; Facco, Monica; Piazza, Francesco; Trentin, Livio; Visentin, Andrea
Contributors: Angotzi, Francesco; Cellini, Alessandro; Ruocco, Valeria; Cavarretta, Chiara Adele; Zatta, Ivan; Serafin, Andrea; Pravato, Stefano; Pagnin, Elisa; Bonaldi, Laura; Frezzato, Federica; Facco, Monica; Piazza, Francesco; Trentin, Livio; Visentin, Andrea
Publisher Information: MDPI
Publication Year: 2024
Collection: Padua Research Archive (IRIS - Università degli Studi di Padova)
Subject Terms: borderline mutated; chronic lymphocytic leukemia; IGHV mutational statu
Description: Chronic lymphocytic leukemia (CLL) exhibits substantial variability in disease course. The mutational status of the B-cell receptor immunoglobulin heavy variable (IGHV) chain is a critical prognostic factor, categorizing patients into mutated (M-IGHV) and unmutated (U-IGHV) groups. Recently, a third subgroup with borderline mutational status (BL-IGHV) has been identified, comprising approximately 5% of CLL cases. This study retrospectively analyzes the outcomes of 30 BL-IGHV mutated patients among a cohort of 653 CLL patients, focusing on time to first treatment (TTFT) and overall survival (OS). BL-IGHV patients had a short TTFT similar to U-IGHV patients (median 30.2 vs. 34 months; p = 0.9). Conversely, the OS of BL-IGHV patients resembled M-IGHV patients (median NR vs. 258 months; p = 1). Despite a similar incidence in unfavorable prognostic factors, the TTFT was shorter compared to other published cohorts. However, striking similarities with other experiences suggest that BL-IGHV mutated patients share common biological characteristics, biased IGHV gene usage and BCR subset frequency. These findings also underscore the need for multicentric efforts aggregating data on BL-IGHV CLL in order to elucidate its disease course and optimize therapeutic approaches for this rare subgroup. Until then, predicting outcomes and optimal management of BL-IGHV CLL will remain challenging.
Document Type: article in journal/newspaper
File Description: ELETTRONICO
Language: English
Relation: info:eu-repo/semantics/altIdentifier/wos/WOS:001191683300001; volume:16; issue:6; journal:CANCERS; https://hdl.handle.net/11577/3509582
DOI: 10.3390/cancers16061095
Availability: https://hdl.handle.net/11577/3509582; https://doi.org/10.3390/cancers16061095
Rights: info:eu-repo/semantics/openAccess ; license:Creative commons ; license uri:http://creativecommons.org/licenses/by/4.0/
Accession Number: edsbas.ECA4E49
Database: BASE