"Hemophagocytic Lymphohistiocytosis after EBV reactivation and ibrutinib treatment in relapsed/refractory Chronic Lymphocytic Leukemia".
Hemophagocytic Lymphohistiocytosis (HLH) is a rare syndrome characterized by ineffective T-cell and NK response. We report the clinical course of a patient with relapsed CLL who developed acute symptoms soon after starting ibrutinib. Hyperpyrexia, splenomegaly, hyperferritinemia, hypertriglyceridemia, cytopenias, and a typical cytokine pattern, i.e. high interleukin (IL)-6, IL10 and IL18, were consistent with a diagnosis of HLH. Coexistent Epstein Barr virus reactivation was documented. Ibrutinib-induced impairment of NK degranulation, associated with EBV reactivation and CLL-related immunodeficiency may have contributed to the development of HLH in our patient.
Hematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, Italy.
Maurizio
M
Cavallari
Hematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, Italy.
Maria
M
Ciccone
Section of Pathology, Department of Oncology and Experimental Biology, University of Ferrara, Ferrara, Italy.
Simonetta
S
Falzoni
Hematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, Italy.
Francesco
F
Cavazzini
Hematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, Italy.
Luca
L
Formigaro
Section of Pathology, Department of Oncology and Experimental Biology, University of Ferrara, Ferrara, Italy.
Francesco
F
Di Virgilio
Section of Microbiology & Medical Genetics, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
Antonella
A
Rotola
Hematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, Italy.
Gian Matteo
GM
Rigolin
Hematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, Italy.
Antonio
A
Cuneo
Anti-cytokine therapy; Chronic Lymphocytic Leukemia; Epstein-barr virus; Hemophagocytic Lymphohisticytosis; Ibrutinib