Recommendations for the management of the haematological and onco-haematological aspects of Gaucher disease

Derralynn Hughes, Maria Domenica Cappellini, Marc Berger, Jan Van Droogenbroeck, Maaike De Fost, Dragana Janic, Theodore Marinakis, Hanna Rosenbaum, Jesús Villarubia, Elena Zhukovskaya, Carla Hollak

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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). 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.

Original languageEnglish
Pages (from-to)676-686
Number of pages11
JournalBritish Journal of Haematology
Volume138
Issue number6
DOIs
Publication statusPublished - Sep 2007

Fingerprint

Gaucher Disease
Monoclonal Gammopathy of Undetermined Significance
Multiple Myeloma
Immunoglobulins
Practice Management
Disease Management
Practice Guidelines
Anemia
Hemoglobins
Therapeutics
Iron
Erythrocytes
Guidelines
Hemorrhage
Morbidity
Incidence

Keywords

  • Clinical haematology
  • Gaucher disease
  • Haematological malignancy

ASJC Scopus subject areas

  • Hematology

Cite this

Recommendations for the management of the haematological and onco-haematological aspects of Gaucher disease. / Hughes, Derralynn; Cappellini, Maria Domenica; Berger, Marc; Van Droogenbroeck, Jan; De Fost, Maaike; Janic, Dragana; Marinakis, Theodore; Rosenbaum, Hanna; Villarubia, Jesús; Zhukovskaya, Elena; Hollak, Carla.

In: British Journal of Haematology, Vol. 138, No. 6, 09.2007, p. 676-686.

Research output: Contribution to journalArticle

Hughes, D, Cappellini, MD, Berger, M, Van Droogenbroeck, J, De Fost, M, Janic, D, Marinakis, T, Rosenbaum, H, Villarubia, J, Zhukovskaya, E & Hollak, C 2007, 'Recommendations for the management of the haematological and onco-haematological aspects of Gaucher disease', British Journal of Haematology, vol. 138, no. 6, pp. 676-686. https://doi.org/10.1111/j.1365-2141.2007.06701.x
Hughes, Derralynn ; Cappellini, Maria Domenica ; Berger, Marc ; Van Droogenbroeck, Jan ; De Fost, Maaike ; Janic, Dragana ; Marinakis, Theodore ; Rosenbaum, Hanna ; Villarubia, Jesús ; Zhukovskaya, Elena ; Hollak, Carla. / Recommendations for the management of the haematological and onco-haematological aspects of Gaucher disease. In: British Journal of Haematology. 2007 ; Vol. 138, No. 6. pp. 676-686.
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