This rare, little known and recently
discovered (1982) affects almost exclusively young women Great Smoky (20
pack-years).
There is a moderate lymphocytosis
with a polyclonal increase in B lymphocytes
A HLA DR7 is associated almost
constantly (see HLA AND CERTAIN DISEASES
and an increase in IgM (see SERUM
IGM) and a particular cytogenetic anomaly. the isochromosome 3q, sometimes with
trisomy 3 in about two-thirds of parents.
A large karyotypic instability (see
KARYOTYPE) is noted and a syndrome of premature condensation of chromatin is
often observed.
The role of tobacco remains unknown
but lymphocytosis decreases during shutdown.
Genetic
abnormalities persist.
This disease is considered benign
but a transformation is possible in non-Hodgkin lymphoma which justifies
prolonged haematological monitoring (see LYMPHOMA).