Waldenstrom Macroglobulinemia, Waldenstrom's Disease
Conditions
Keywords
Waldenstrom's Disease, Waldenstrom Macroglobulinemia
Brief summary
Waldenström Macroglobulinemia (WM) is defined by a bone marrow lymphoplasmacytic infiltration and the presence of a monoclonal immunoglobulin M (IgM) in blood. This chronic lymphoproliferative disorder requires treatment only in case of symptoms, according to accurate criteria described during the second Workshop on WM i.e. in case of cytopenia, bulky organomegaly, immunological or physicochemical consequences of the presence of IgM in circulating blood. A MYD88 mutation, typically a MYD88(L265P), is found in 90% of WM patients. Other gene abnormalities have been observed, the most frequent is a mutation in the CXCR4 gene. Overall, gene mutations in WM involve only a limited number of signalling pathways, yielding the activation of NFkB, namely : the TLR and MYD88 pathway (with an activation of NFkB and BTK in case of MYD88(L265P) mutation), the BCR pathway (involving btk and associated with activations of both NFkB, and erk akt pathway) and the CXCR4 pathway (CXCR4 is a receptor of CXCL12, it is also associated with activations of ERK/MAPK and PI3K). Abnormalities of some of genes, such as TP53, of the expression of the protein CXCL13 and genes involved in the interleukin 6 secretion have been associated with some clinical characteristics. The purpose of this project is to define the prognostic role of the detection of circulating tumoral DNA (ctDNA) at the end of treatment for the progression/relapse risk within the first 3 years after the first 6 months of treatment.
Interventions
BIOLOGICALbone marrow sample
8 to 13 ml bone marrow sample for frozen storage within one of the 5 certified biobank of the canceropole area.
BIOLOGICALblood sample
20 ml blood sample for frozen storage within one of the 5 certified biobank of the canceropole area.
Sponsors
Centre Henri Becquerel
University Hospital, Caen
University Hospital, Lille
Centre Hospitalier de Lens
Centre Hospitalier Universitaire, Amiens
Study design
Allocation
NON_RANDOMIZED
Intervention model
SINGLE_GROUP
Primary purpose
OTHER
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Patient with WM according to diagnostic criteria * Patients with WM followed in one of the centre of North-Western region. * Patients requiring first-line or subsequent-line therapy * Patients agreement for giving informed consent. * Social insurance system affiliation
Exclusion criteria
* Patients with another chronic B-cell malignancy * patients with other lymphoplasmacytic proliferations * patients with marginal zone lymphoma. * Patients with WM and histologic transformation * Absence of informed consent
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| association between circulating tumoral DNA detection and progression-free survival | 3 years | To define the prognostic role of the detection of circulating tumoral DNA (ctDNA) at the end of treatment for the progression/relapse risk within the first 3 years after the first 6 months of treatment. |
Countries
France
Contacts
Primary ContactPierre MOREL, MD
Outcome results
None listed