Ibrutinib treatment of a patient with relapsing chronic lymphocytic leukemia and sustained remission of Richter syndrome

Tumori 2017; 103(Suppl. 1)

Article Type: CASE REPORT



Albi, Elisa Baldoni, Stefano Aureli, Patrizia Dorillo, Erica Del Papa, Beatrice Ascani, Stefano Di Ianni, Mauro Falzetti, Franca Sportoletti, Paolo



Richter syndrome (RS) is a rare event in chronic lymphocytic leukemia (CLL) that is influenced by biological factors and prior CLL treatments. Ibrutinib is a Bruton tyrosine kinase inhibitor that has shown remarkable efficacy in CLL; however, little is known about its relationship to RS. We report a case of ibrutinib efficacy against CLL in a patient with prolonged remission of RS.


The patient was diagnosed with CLL in 2003. Biological findings at onset included absent ZAP70 expression, mutated IGVH, and NOTCH1 mutation. He was treated with FCR with partial response. In 2013, he progressed to RS, not clonally related to the underlying CLL. The patient was treated with anthracycline- and platinum-based regimens, obtaining a complete remission. After 3 years, he presented a CLL progression with worsening lymphocytosis, anemia, thrombocytopenia, increased splenomegaly, and lymphadenopathies. Positron emission tomography-computed tomography scan excluded pathologic uptake. Thus, he was started on ibrutinib.


At 12 months’ follow-up, we observed white blood cell normalization, increased hemoglobin and platelet levels, disappearance of lymphadenopathy, and spleen size reduction. Therapy was well-tolerated with no evidence of RS.


This case demonstrates sustained RS remission in a patient with CLL under ibrutinib therapy, thus improving our knowledge on the use of this new drug in CLL and beyond.

Article History


Financial support: Supported by grants from the Associazione Italiana per la Ricerca sul Cancro (MFAG 2015 Id.17442 to P.S.) and Ministero dell’Istruzione, dell’Università e della Ricerca (Programma SIR–RBSI14GPBL to P.S.).
Conflict of interest: None of the authors has conflict of interest with this submission.

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