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Transformed Waldenström macroglobulinaemia: clinical presentation and outcome. A multi-institutional retrospective study of 77 cases from the French Innovative Leukemia Organization (FILO).
- Source :
-
British journal of haematology [Br J Haematol] 2017 Nov; Vol. 179 (3), pp. 439-448. Date of Electronic Publication: 2017 Aug 02. - Publication Year :
- 2017
-
Abstract
- Histological transformation (HT) to diffuse large B-cell lymphoma (DLBCL) is a rare and poorly reported complication of Waldenström macroglobulinaemia (WM). We performed a retrospective study of 77 WM patients with biopsy-proven transformation to DLBCL. The median time from WM diagnosis to HT was 4·6 years and 16 patients (21%) had never been treated for WM. At HT, extranodal sites were observed in 91% of patients with a rather high incidence of central nervous system, cutaneous or testicular involvement. Fluorodeoxyglucose-positron emission tomography was performed in half of the patients and the median maximum standardized uptake value was 15 for transformed disease. More than 80% of cases with available data for assessment by the Hans' algorithm harboured a non-germinal centre B-cell phenotype. First-line treatment for transformation consisted of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)-like regimen in 85% of patients. The overall response rate after first-line treatment was 61% and the median overall survival was only 16 months for the entire cohort. Time to transformation above 5 years (P = 0·0004) and elevated LDH (P = 0·02) were associated with worse outcome. Based on these findings, HT should be considered and lead to a biopsy in WM patients presenting with extranodal involvement, elevated LDH and constitutional symptoms. The optimal therapeutic approaches remain to be defined.<br /> (© 2017 John Wiley & Sons Ltd.)
- Subjects :
- Adult
Aged
Aged, 80 and over
Antibodies, Monoclonal, Murine-Derived therapeutic use
Antineoplastic Combined Chemotherapy Protocols therapeutic use
Biomarkers, Tumor blood
Biopsy
Cyclophosphamide therapeutic use
Doxorubicin therapeutic use
Female
Fluorodeoxyglucose F18
Humans
L-Lactate Dehydrogenase blood
Lymphoma, Large B-Cell, Diffuse diagnostic imaging
Lymphoma, Large B-Cell, Diffuse drug therapy
Male
Middle Aged
Positron-Emission Tomography methods
Prednisone therapeutic use
Prognosis
Retrospective Studies
Risk Factors
Rituximab
Survival Analysis
Tomography, X-Ray Computed
Treatment Outcome
Vincristine therapeutic use
Waldenstrom Macroglobulinemia diagnostic imaging
Cell Transformation, Neoplastic pathology
Lymphoma, Large B-Cell, Diffuse pathology
Waldenstrom Macroglobulinemia pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1365-2141
- Volume :
- 179
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- British journal of haematology
- Publication Type :
- Academic Journal
- Accession number :
- 28770576
- Full Text :
- https://doi.org/10.1111/bjh.14881