Recommendations for the management of the haematological and onco-haematological aspects of Gaucher disease.
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.
Hughes
Derralynn
D
Department of Academic Haematology, Royal Free Hospital and University College Medical School, London, UK. d.hughes@medsch.ucl.ac.uk
Cappellini
Maria Domenica
MD
Berger
Marc
M
Van Droogenbroeck
Jan
J
de Fost
Maaike
M
Janic
Dragana
D
Marinakis
Theodore
T
Rosenbaum
Hanna
H
Villarubia
Jesús
J
Zhukovskaya
Elena
E
Hollak
Carla
C
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