Persistent polyclonal lymphocytosis with binucleated B lymphocytes: a genetic predisposition.
Persistent lymphocytosis is usually associated with a malignant lymphoproliferative disease (MLPD). We report six female patients presenting a chronic, moderate lymphocytosis of 2-16 years duration with atypical binucleated lymphocytes on peripheral blood smears. Further investigation showed a polyclonal increase in serum IgM and HLA-DR7 phenotype in all patients. The B cells were polyclonal because Southern hybridization of DNA and polymerase chain reaction failed to demonstrate a clonal rearrangement of immunoglobulin heavy chain genes. Peripheral blood examination showed binucleated lymphocytes in a family member of two of the cases; taken together with the association with HLA-DR7 these data suggest a genetic predisposition. The identification of this benign syndrome is important in order to prevent its misdiagnosis as a MLPD.
Troussard
X
X
Laboratoire d'Hématologie, Hôpital Necker-Enfants Malades, Paris, France.
Valensi
F
F
Debert
C
C
Maynadie
M
M
Schillinger
F
F
Bonnet
P
P
Macintyre
E A
EA
Flandrin
G
G
D000328:Adult; D000954:Antigens, Surface / Q000097:blood; D001402:B-Lymphocytes / Q000473:pathology*; D001483:Base Sequence; D002908:Chronic Disease; D004198:Disease Susceptibility; D005260:Female; D005500:Follow-Up Studies; D005803:Genes, Immunoglobulin; D015809:HLA-DR7 Antigen / Q000097:blood; D006801:Humans; D016130:Immunophenotyping / Q000379:methods; D008218:Lymphocytosis / Q000097:blood / Q000235:genetics* / Q000276:immunology; D008875:Middle Aged; D008969:Molecular Sequence Data; D016133:Polymerase Chain Reaction
D000954:Antigens, Surface; D015809:HLA-DR7 Antigen