“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, hypertriglyceridemi...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2017-01-01
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| Series: | Leukemia Research Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2213048916300437 |
| _version_ | 1819109803021041664 |
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| author | Maurizio Cavallari Maria Ciccone Simonetta Falzoni Francesco Cavazzini Luca Formigaro Francesco Di Virgilio Antonella Rotola Gian Matteo Rigolin Antonio Cuneo |
| author_facet | Maurizio Cavallari Maria Ciccone Simonetta Falzoni Francesco Cavazzini Luca Formigaro Francesco Di Virgilio Antonella Rotola Gian Matteo Rigolin Antonio Cuneo |
| author_sort | Maurizio Cavallari |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-22T03:31:37Z |
| format | Article |
| id | doaj.art-29b95a9ac199491caa6c0058a925b573 |
| institution | Directory Open Access Journal |
| issn | 2213-0489 |
| language | English |
| last_indexed | 2024-12-22T03:31:37Z |
| publishDate | 2017-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Leukemia Research Reports |
| spelling | doaj.art-29b95a9ac199491caa6c0058a925b5732022-12-21T18:40:29ZengElsevierLeukemia Research Reports2213-04892017-01-017C111310.1016/j.lrr.2017.01.002“Hemophagocytic Lymphohistiocytosis after EBV reactivation and ibrutinib treatment in relapsed/refractory Chronic Lymphocytic Leukemia”Maurizio Cavallari0Maria Ciccone1Simonetta Falzoni2Francesco Cavazzini3Luca Formigaro4Francesco Di Virgilio5Antonella Rotola6Gian Matteo Rigolin7Antonio Cuneo8Hematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, ItalyHematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, ItalySection of Pathology, Department of Oncology and Experimental Biology, University of Ferrara, Ferrara, ItalyHematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, ItalyHematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, ItalySection of Pathology, Department of Oncology and Experimental Biology, University of Ferrara, Ferrara, ItalySection of Microbiology & Medical Genetics, Department of Medical Sciences, University of Ferrara, Ferrara, ItalyHematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, ItalyHematology Section, Azienda Ospedaliero-Universitaria Arcispedale S. Anna, Ferrara, ItalyHemophagocytic 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.http://www.sciencedirect.com/science/article/pii/S2213048916300437Hemophagocytic LymphohisticytosisChronic Lymphocytic LeukemiaEpstein-barr virusIbrutinibAnti-cytokine therapy |
| spellingShingle | Maurizio Cavallari Maria Ciccone Simonetta Falzoni Francesco Cavazzini Luca Formigaro Francesco Di Virgilio Antonella Rotola Gian Matteo Rigolin Antonio Cuneo “Hemophagocytic Lymphohistiocytosis after EBV reactivation and ibrutinib treatment in relapsed/refractory Chronic Lymphocytic Leukemia” Leukemia Research Reports Hemophagocytic Lymphohisticytosis Chronic Lymphocytic Leukemia Epstein-barr virus Ibrutinib Anti-cytokine therapy |
| title | “Hemophagocytic Lymphohistiocytosis after EBV reactivation and ibrutinib treatment in relapsed/refractory Chronic Lymphocytic Leukemia” |
| title_full | “Hemophagocytic Lymphohistiocytosis after EBV reactivation and ibrutinib treatment in relapsed/refractory Chronic Lymphocytic Leukemia” |
| title_fullStr | “Hemophagocytic Lymphohistiocytosis after EBV reactivation and ibrutinib treatment in relapsed/refractory Chronic Lymphocytic Leukemia” |
| title_full_unstemmed | “Hemophagocytic Lymphohistiocytosis after EBV reactivation and ibrutinib treatment in relapsed/refractory Chronic Lymphocytic Leukemia” |
| title_short | “Hemophagocytic Lymphohistiocytosis after EBV reactivation and ibrutinib treatment in relapsed/refractory Chronic Lymphocytic Leukemia” |
| title_sort | hemophagocytic lymphohistiocytosis after ebv reactivation and ibrutinib treatment in relapsed refractory chronic lymphocytic leukemia |
| topic | Hemophagocytic Lymphohisticytosis Chronic Lymphocytic Leukemia Epstein-barr virus Ibrutinib Anti-cytokine therapy |
| url | http://www.sciencedirect.com/science/article/pii/S2213048916300437 |
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