Response to ibrutinib of a refractory IgA lymphoplasmacytic lymphoma carrying the MYD88 L265P gene mutation

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Francesca Maria Quaglia
Gian Matteo Rigolin *
Elena Saccenti
Massimo Negrini
Eleonora Volta
Melissa Dabusti
Maria Ciccone
Antonio Urso
Michele Laudisi
Antonio Cuneo
(*) Corresponding Author:
Gian Matteo Rigolin | rglgmt@unife.it

Abstract

In 2014 a 66 year-old woman presented with anemia and an IgAk monoclonal spike. Bone marrow (BM) biopsy showed 80% lymphocytes and lymphoplasmacytoid cells. Computed Tomography (CT) scan documented neither adenopathy nor splenomegaly. Diagnosis of IgA lymphoplasmacytic lymphoma was made. After three lines of treatment, progressive disease with adenopathies, splenomegaly and ascites were documented on a CT scan. Our patient developed thrombocytopenia, transfusion-dependent anemia and clinical deterioration. We performed genetic studies of peripheral blood lymphoctyes with NGS approach. Given the identification of MYD88 L265P mutation, in February 2018 our patient started ibrutinib. Hb and PLT improved from day +35. In July 2018 no ascites and 50% reduction of adenopathies and spleen were shown on a CT scan. In April 2019 the patient was still on ibrutinib with transfusion independence and good performance status.


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Author Biographies

Francesca Maria Quaglia, University of Verona/University of Ferrara

Stem Cell Research Laboratory, Section of Hematology, Department of Medicine of the Verona University.

Hematology Section, Department of Medical Sciences, Azienda Ospedaliero-Universitaria Arcispedale S.Anna, University of Ferrara.

Gian Matteo Rigolin, Azienda Ospedaliero-Universitaria Arcispedale S.Anna, University of Ferrara

Hematology Section, Department of Medical Sciences.

Elena Saccenti, Azienda Ospedaliero-Universitaria Arcispedale S.Anna, University of Ferrara

Hematology Section, Department of Medical Sciences.

Massimo Negrini, University of Ferrara

Department of Morphology, Surgery and Experimental Medicine.

Eleonora Volta, Azienda Ospedaliero-Universitaria Arcispedale S.Anna, University of Ferrara

Hematology Section, Department of Medical Sciences.

Maria Ciccone, Azienda Ospedaliero-Universitaria Arcispedale S.Anna, University of Ferrara

Hematology Section, Department of Medical Sciences.

Antonio Urso, Azienda Ospedaliero-Universitaria Arcispedale S.Anna, University of Ferrara

Hematology Section, Department of Medical Sciences.

Michele Laudisi, Azienda Ospedaliero-Universitaria Arcispedale S.Anna, University of Ferrara

Hematology Section, Department of Medical Sciences.

Antonio Cuneo, Azienda Ospedaliero-Universitaria Arcispedale S.Anna, University of Ferrara

Hematology Section, Department of Medical Sciences.