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Saint-André-lez-Lille, France

Cherin P.,Medecine Interne | Rose C.,Hematologie | De Roux-Serratrice C.,Medecine Interne | Tardy D.,Actelion Pharmaceuticals | And 9 more authors.
Journal of Inherited Metabolic Disease | Year: 2010

Background: Gaucher disease (GD), the most prevalent inherited lysosomal storage disorder, is caused by deficient glucocerebrosidase activity. Type 1 GD (GD1), the most common variant, is classically considered non-neuronopathic. Methods: We performed a national cross-sectional observational survey-the French Observatoire on Gaucher Disease (FROG)-in patients with GD1 between March 2005 and September 2006. The study included all patients over 18 years of age with confirmed GD1 who attended participating centers for regular follow-up. Results: One hundred and five patients were included, in whom we studied the prevalence and characteristics of relevant neurological symptoms associated with the neuraxis. Of these, 51 (49%) GD1 patients presented at least one neurological symptom. Four patients (4%) had Parkinson disease and 22 (21%) presented with at least one parkinsonian sign or at least one sign frequently associated with Parkinson disease. Five patients (5%) had a previous diagnosis of peripheral neuropathy. Other central nervous system symptoms were recorded in 20 (19%) patients and other peripheral nervous system symptoms in 39 (37%) patients. Conclusions: These data challenge the current classification of GD, and suggest that the three forms of GD each involve a different profile of neurological manifestations. © 2010 SSIEM and Springer. Source


Hunfeld N.,Ziekenhuisapotheker in Opleiding | Evers D.,Interne Geneeskunde | Van Hest R.,Ziekenhuisapotheker in Opleiding | Wijermans P.,Hematologie
Pharmaceutisch Weekblad | Year: 2010

Objective: This case report describes renal failure in a 71 year old male with multiple myeloma, stage IIA, after administration of bortezomib and erythromycin. We considered the possibility of an interaction between bortezomib and erythromycin. Design and methods: Description of the case and literature search. Results: During the first cycle of bortezomib, the patient showed a decrease in renal clearance (creatinin increased from 150 to 370 μmol/l). Renal function recovered within a week. Bortezomib was then continued at a reduced dose of 50%. Two days before the fourth administration, the patient developed fever. Erythromycin was started (500 mg p.o. four times daily), creatinin was still 150 μmol/l. One week later, the patient developed renal failure (creatinin 541 μmol/l) and was diagnosed with tubulo-interstitial nephritis caused by bortezomib toxicity likely based on an interaction with erythromycin. There is no information about this interaction in literature, but its occurrence can be explained by effects on CYP3A4 metabolism. Erythromycin is a moderate CYP3A4 inhibitor and substrate and bortezomib is a CYP3A4 substrate. Conclusion: We recommend frequent monitoring (serum creatinin on day 3/4 and day 7/8) of renal function in patients during and after administration of bortezomib in combination with a CYP3A4 inhibitor. Source


Ham J.C.,Isala Klinieken | Ruijs G.J.H.M.,Laboratorium voor Medische Microbiologie en Infectieziekten | Van Marwijk Kooy M.,Hematologie
Nederlands Tijdschrift voor Geneeskunde | Year: 2013

A 43-year-old patient with recurrent acute myeloid leukemia (AML) was treated with high-dose cytarabine. After two weeks of neutropenic fever, multiple cutaneous nodules appeared. Histopathological examination of a skin biopsy showed a mycosis and Fusarium solani was cultured. Despite antimycotic therapy, the patient died due to complications of his AML treatment. Source


Robin M.,Hematology Bone Marrow Transplantation | Giannotti F.,Eurocord International Registry | Deconinck E.,University of Franche Comte | Mohty M.,Hematologie | And 23 more authors.
Biology of Blood and Marrow Transplantation | Year: 2014

To determine whether umbilical cord blood transplantation (UCBT) is an alternative cure for myelofibrosis (MF), we evaluated 35 UCBTs reported to Eurocord. Seven patients had secondary acute myeloid leukemia (AML) at UCBT, and median age at UCBT was 54 years. Twenty-four patients received a reduced-intensity conditioning (RIC) regimen, and 17 of 35 patients received total body irradiation (2 to 12 Gy)-fludarabine-cyclophosphamide (TCF) conditioning. The median follow-up was 24 months. The cumulative incidence of neutrophil recovery at 60 days was 80%. Fifteen patients relapsed after UCBT. The 2-year overall survival and event-free-survival (EFS) rates were 44% and 30%, respectively. All patients given TCF achieved neutrophil and platelet recovery, and the use of TCF was associated with superior EFS in the RIC population (44% versus 0%, P= .001). Patients with transformation to AML had similar outcomes to patients with less advanced stages. In conclusion, despite graft failure remaining a major concern, the role of UCBT in the management of MF, especially using RIC TCF-based regimens, deserves further investigation to improve results. © 2014 American Society for Blood and Marrow Transplantation. Source


Lengline E.,Hematologie | Darmon M.,Reanimation | Azoulay E.,Reanimation | Gall J.-R.L.E.,LAcademie nationale de medecine
Bulletin de l'Academie Nationale de Medecine | Year: 2015

Overall prognosis of cancer or haematological has dramatically decreased over the last decades. Thus advances regarding cancer or haematological treatment, improved knowledge of usual complications and of their pathophysiology and changes in ICU admission policy and management are among factors which participated to the overall prognostic changes. Tyrosine-Kinase inhibitors in patients with chronic myeloid leukemia and anti-CD20 antibodies in patients with non-hodgkin's lymphoma were among the first success of targeted therapies. These success stories have been followed by others and no less than 13 targeted therapies were available for cancer patients in December 2013. Additionally, pathophysiology of complication is better understood and prognostic impact of organ failure better apprehended. Standardized diagnostic criteria of tumor lysis syndrome along with improved understanding of short-term and long term influence of acute kidney injury (AKI) in this setting have led to specific management strategies focusing on prevention. In non-malignant haematological diseases, pathophysiological processes leading to thrombotic thrombocytopenic purpura or atypical haemolytic and uremic syndrome are now better understood leading to additional therapeutic options. Last, diversification of ICU admission policies may help in taking into account uncertainties, therapeutic advances and patients' autonomy. This review will give an overview of these recent advances. Source

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