Volumen: 20 # Number : 3
Publication Date : Septiembre - Diciembre Year: 2016
Clinical and biological factors related to the
transformation of chronic lymphocytic leukemia
into diffuse large B-cell lymphoma
Authors: Fuente ML
Abstract: Chronic lymphocytic leukemia can suffer transformation
into a high grade lymphoma during its
evolution, frequently into a diffuse large B-cell
lymphoma clonally related. That is known as
Richter’s syndrome. A biopsy is needed to diagnose
and it may be guided with a PET-CT, with high
negative predictive value. Different factors have
been associated with the risk of transformation:
bulky lymphadenopathy, RAI stage III-IV, unmutated
IgHV, some stereotyped B-cell receptors, positive
expression of CD38, ZAP70, CD49d, somatic
mutation in CDKN2A and NOTCH1 genes, genetic
polymorphisms and CLL treatment. It seems that the
lymph node environment favors the transformation.
Three different genetic pathways have been
described involved in CLL transformation involved
in transformation of CLL, and TP53 gen disruptions
are the most frequent genetic acquired alteration. The
aggressiveness of this disease and its low response
to therapy might be associated with polymorphisms.
Key words: Richter Syndrome,
Chronic lymphocytic leukemia,
Cell transformation.
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