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Published on Feb 2, 2021
Medicine
14. Richter Syndrome
Richter Syndrome
T
ransformation of
CLL
into an aggressive large B-cell lymphoma
(seen in 3-10% of cases)
Inactivation of
TP53 and of CDKN2A
is a main mechanism in the transformation to Richter syndrome
Manifests with weight loss, fevers, night sweats, muscle wasting, (i.e., B symptoms) and increasing hepatosplenomegaly and lymphadenopathy.
Treatment remains challenging and prognosis poor
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