title: Recommendations for the management of the haematological and onco-haematological aspects of Gaucher disease.
abstract: Current knowledge of the haematological and onco-haematological complications of type 1 Gaucher disease has been reviewed with the aim of identifying best clinical practice for treatment and disease management. It was concluded that: (i) Awareness of typical patterns of cytopenia can help clinicians distinguish haematological co-morbidities. (ii) Red blood cell studies and complete iron metabolism evaluation at baseline are recommended. (iii) Haemoglobin levels defining anaemia should be raised and used in Gaucher disease treatment and monitoring. (iv) Surgeons should be aware of potential bleeding complications during surgery in Gaucher patients. The higher incidence of multiple myeloma in Gaucher disease suggests that Gaucher patients should have their immunoglobulin profile determined at diagnosis and monitored every 2 years (patients <50 years) or every year (patients >50 years). If monoclonal gammopathy of undetermined significance (MGUS) is found, general MGUS guidelines should be followed. Future studies should focus on the utility of early treatment to prevent immunoglobulin abnormalities and multiple myeloma.
authors.affiliation: Department of Academic Haematology, Royal Free Hospital and University College Medical School, London, UK. d.hughes@medsch.ucl.ac.uk
authors.forename: Derralynn
authors.initials: D
authors.lastname: Hughes
authors.forename: Maria Domenica
authors.initials: MD
authors.lastname: Cappellini
authors.forename: Marc
authors.initials: M
authors.lastname: Berger
authors.forename: Jan
authors.initials: J
authors.lastname: Van Droogenbroeck
authors.forename: Maaike
authors.initials: M
authors.lastname: de Fost
authors.forename: Dragana
authors.initials: D
authors.lastname: Janic
authors.forename: Theodore
authors.initials: T
authors.lastname: Marinakis
authors.forename: Hanna
authors.initials: H
authors.lastname: Rosenbaum
authors.forename: Jesús
authors.initials: J
authors.lastname: Villarubia
authors.forename: Elena
authors.initials: E
authors.lastname: Zhukovskaya
authors.forename: Carla
authors.initials: C
authors.lastname: Hollak
mesh_terms: D032921:Consensus; D005060:Europe; D005776:Gaucher Disease / Q000150:complications / Q000276:immunology / Q000628:therapy*; D019337:Hematologic Neoplasms / Q000150:complications / Q000175:diagnosis; D006405:Hematology; D006801:Humans; D009101:Multiple Myeloma / Q000150:complications / Q000175:diagnosis; D010265:Paraproteinemias / Q000150:complications / Q000175:diagnosis