Premium
B‐cell repertoire and clonal analysis in unaffected first degree relatives in familial chronic lymphocytic leukaemia kindred
Author(s) -
Abbasi Fatima,
Longo Nancy S.,
Lipsky Peter E.,
Raveche Elizabeth,
Schleinitz Therese A.,
StetlerStevenson Maryalice,
Caporaso Neil,
Marti Gerald
Publication year - 2007
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2007.06857.x
Subject(s) - ighv@ , cd5 , lymphocytosis , biology , proband , immunology , monoclonal , chronic lymphocytic leukemia , genetics , flow cytometry , antibody , gene , leukemia , monoclonal antibody , mutation
Summary Monoclonal B cell lymphocytosis (MBL) was detected in four unaffected first‐degree relatives (FDR) in a familial chronic lymphocytic leukaemia (CLL) kindred. The proband remains untreated and two male siblings have died. The four unaffected siblings have been followed for a five‐year period. All four FDR developed a kappa + CD5 + MBL detected by flow cytometry. Poymerase chain reaction (PCR) for IGHV rearrangement showed evidence of oligoclonality in three of these individuals. Single cell PCR of flow cytometric sorted kappa + cells combined with Ig kappa light chain gene sequencing revealed further evidence of monoclonality in two of these individuals. Three of these individuals all showed evidence of hyper‐somatic mutations. The B‐cell repertoire in unaffected FDR in familial CLL offers a new area to investigate the interface between the immune system and lymphoid neoplasm.