ALK-positive histiocytosis associated with chronic lymphocytic leukaemia/small lymphocytic lymphoma: a multitarget respo
- PDF / 952,531 Bytes
- 5 Pages / 595.276 x 790.866 pts Page_size
- 67 Downloads / 197 Views
BRIEF REPORT
ALK-positive histiocytosis associated with chronic lymphocytic leukaemia/small lymphocytic lymphoma: a multitarget response under ibrutinib Charlotte Syrykh 1 & Loïc Ysebaert 2,3,4,5 & Sarah Péricart 1 & Solène M. Evrard 1,2 & Fabienne Meggetto 2,5 & Salim Kanoun 6 & Pierre Brousset 1,2,5 & Camille Laurent 1,2,4,5 Received: 1 May 2020 / Revised: 6 September 2020 / Accepted: 23 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract ALK-positive histiocytosis is a recently described entity with few reported cases in literature. Herein, we report an unusual case of ALK-positive histiocytosis showing an Erdheim-Chester disease (ECD)-like presentation, occurring in a 37-year-old woman with a 2-year history of chronic lymphocytic leukaemia (CLL). Our CLL patient relapsed 6 months after the end of fludarabine, cyclophosphamide and rituximab frontline therapy and complained of lower limb pains. A bone marrow biopsy was performed and showed concomitant CLL/small lymphocytic lymphoma and ALK-positive histiocytosis with an identical immunoglobulin heavy-chain gene rearrangement in both neoplasms, suggesting clonal relationship. After 4 years under ibrutinib therapy, our patient remains free of both diseases. This report extends the spectrum of composite hematolymphoid neoplasms and shows that ALK rearrangement should be considered in all histiocytosis subtypes. Moreover, both tumours eradication under ibrutinib suggests that BTK inhibitors may also be effective in histiocytic neoplasms. Keywords ALK-positive histiocytosis . Erdheim-Chester disease . Chronic lymphocytic leukaemia . ALK . Ibrutinib
Introduction Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00428-020-02937-y) contains supplementary material, which is available to authorized users. * Camille Laurent [email protected] 1
Department of Pathology, Toulouse University Hospital Center, Cancer Institute University of Toulouse-Oncopole, 1 avenue Irène Joliot-Curie, 31059 Toulouse CEDEX 9, France
2
INSERM UMR1037 Cancer Research Center of Toulouse (CRCT), ERL 5294 National Center for Scientific Research (CNRS), University of Toulouse III Paul-Sabatier, Toulouse, France
3
Departement of Hematology, Toulouse University Hospital Center, Cancer Institute University of Toulouse-Oncopole, Toulouse, France
4
Programme Hospitalo-Universitaire en Cancérologie CAPTOR, Toulouse, France
5
Institut Carnot Lymphome CALYM, Laboratoire d’Excellence ‘TOUCAN’, Toulouse, France
6
Department of Nuclear Medicine, Toulouse University Hospital Center, Cancer Institute University of Toulouse-Oncopole, Toulouse, France
Histiocytoses are rare diseases defined by the accumulation, in various tissues, of mononuclear phagocytes such as dendritic cells or macrophage-derived cells. A revised histiocytosis classification system was proposed in 2016 by Emile et al., separating the broad spectrum of histiocytoses into five groups [1]. Among them, the Langerhans related (L) group includes Lang
Data Loading...
