ALK-positive histiocytosis associated with chronic lymphocytic leukaemia/small lymphocytic lymphoma: a multitarget response under ibrutinib.

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.

• Authors: Charlotte Syrykh, Loïc Ysebaert, Sarah Péricart, Solène M Evrard, Fabienne Meggetto, Salim Kanoun, Pierre Brousset, Camille Laurent
• Journal: Virchows Archiv : an international journal of pathology (Virchows Arch) Vol. 478 Issue 4 Pg. 779-783 (Apr 2021) ISSN: 1432-2307 [Electronic] Germany
• PMID: 33011863 (Publication Type: Case Reports, Journal Article)

Chemical References

• Biomarkers
• Piperidines
• Protein Kinase Inhibitors
• ibrutinib
• ALK protein, human
• Anaplastic Lymphoma Kinase
• Adenine

Topics

• Adenine (analogs & derivatives, therapeutic use)
• Adult
• Anaplastic Lymphoma Kinase (metabolism)
• Biomarkers (metabolism)
• Female
• Histiocytosis (diagnosis, drug therapy, etiology, metabolism)
• Humans
• Leukemia, Lymphocytic, Chronic, B-Cell (complications, drug therapy)
• Piperidines (therapeutic use)
• Protein Kinase Inhibitors (therapeutic use)