1 Stem Cell Research Laboratory, Section of Hematology, Department of Medicine, University of Verona, Verona, Italy.
2 Hematology Section, Department of Medical Sciences, Azienda Ospedaliero-Universitaria, Arcispedale S.Anna, University of Ferrara, Ferrara, Italy.
3 Department of Morphology, Surgery and Experimental Medicine, University of Ferrara, Ferrara, Italy.
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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 lymphocytes with the NGS approach. Given the
identification of MYD88 L265P mutation, in February 2018 our patient
started ibrutinib off-label. 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.
1. The diagram illustrates modifications in hemoglobin (Hb) and IgAk
monoclonal spike (left Y axis), platelets (PLT) and symptoms (right Y
axis) by treatment in our patient. Symptom Scale: 0–100-point scale,
based on patient’s reported symptoms. Higher scores indicate more
RCD: Rituximab-Cyclophosphamide-Dexamethasone; CHOP: Cyclophosphamide-Doxorubicin-Vincristine-Prednisone. Red arrow: initiation of ibrutinib.