Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment
Histological transformation (HT) to an aggressive lymphoma results from a rare evolution of Waldenström macroglobulinemia (WM). A higher incidence of transformation events has been reported in <i>MYD88</i> wild-type WM patients. HT in WM can be histologically heterogeneous, although the...
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MDPI AG
2022-10-01
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| author | Eric Durot Cécile Tomowiak Elise Toussaint Pierre Morel Dipti Talaulikar Prashant Kapoor Jorge J. Castillo Alain Delmer |
| author_facet | Eric Durot Cécile Tomowiak Elise Toussaint Pierre Morel Dipti Talaulikar Prashant Kapoor Jorge J. Castillo Alain Delmer |
| author_sort | Eric Durot |
| collection | DOAJ |
| description | Histological transformation (HT) to an aggressive lymphoma results from a rare evolution of Waldenström macroglobulinemia (WM). A higher incidence of transformation events has been reported in <i>MYD88</i> wild-type WM patients. HT in WM can be histologically heterogeneous, although the diffuse large B-cell lymphoma of activated B-cell subtype is the predominant pathologic entity. The pathophysiology of HT is largely unknown. The clinical suspicion of HT is based on physical deterioration and the rapid enlargement of the lymph nodes in WM patients. Most transformed WM patients present with elevated serum lactate dehydrogenase (LDH) and extranodal disease. A histologic confirmation regarding the transformation to a higher-grade lymphoma is mandatory for the diagnosis of HT, and the choice of the biopsy site may be dictated by the findings of the <sup>18</sup>fluorodeoxyglucose-positron emission tomography/computed tomography. The prognosis of HT in WM is unfavorable, with a significantly inferior outcome compared to WM patients without HT. A validated prognostic score based on 3 adverse risk factors (elevated LDH, platelet count < 100 × 10<sup>9</sup>/L and any previous treatment for WM) stratifies patients into 3 risk groups. The most common initial treatment used is a chemo-immunotherapy (CIT), such as R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone). The response duration is short and central nervous system relapses are frequent. Whether autologous stem cell transplantation could benefit fit patients responding to CIT remains to be studied. |
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| issn | 2673-6357 |
| language | English |
| last_indexed | 2024-03-09T16:21:24Z |
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| series | Hemato |
| spelling | doaj.art-16741abba5384a3794c2bd6533ac4da02023-11-24T15:12:43ZengMDPI AGHemato2673-63572022-10-013465066210.3390/hemato3040044Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and TreatmentEric Durot0Cécile Tomowiak1Elise Toussaint2Pierre Morel3Dipti Talaulikar4Prashant Kapoor5Jorge J. Castillo6Alain Delmer7Department of Hematology, University Hospital of Reims and UFR Médecine, 51092 Reims, FranceDepartment of Hematology and CIC U1402, University Hospital of Poitiers, 86000 Poitiers, FranceDepartment of Hematology, University Hospital of Strasbourg, 67200 Strasbourg, FranceDepartment of Hematology, University Hospital of Amiens, 80480 Amiens, FranceDepartment of Hematology, Canberra Health Services, College of Health and Medicine, Australian National University, Canberra, ACT 2600, AustraliaDivision of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN 55901, USABing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA 02459, USADepartment of Hematology, University Hospital of Reims and UFR Médecine, 51092 Reims, FranceHistological transformation (HT) to an aggressive lymphoma results from a rare evolution of Waldenström macroglobulinemia (WM). A higher incidence of transformation events has been reported in <i>MYD88</i> wild-type WM patients. HT in WM can be histologically heterogeneous, although the diffuse large B-cell lymphoma of activated B-cell subtype is the predominant pathologic entity. The pathophysiology of HT is largely unknown. The clinical suspicion of HT is based on physical deterioration and the rapid enlargement of the lymph nodes in WM patients. Most transformed WM patients present with elevated serum lactate dehydrogenase (LDH) and extranodal disease. A histologic confirmation regarding the transformation to a higher-grade lymphoma is mandatory for the diagnosis of HT, and the choice of the biopsy site may be dictated by the findings of the <sup>18</sup>fluorodeoxyglucose-positron emission tomography/computed tomography. The prognosis of HT in WM is unfavorable, with a significantly inferior outcome compared to WM patients without HT. A validated prognostic score based on 3 adverse risk factors (elevated LDH, platelet count < 100 × 10<sup>9</sup>/L and any previous treatment for WM) stratifies patients into 3 risk groups. The most common initial treatment used is a chemo-immunotherapy (CIT), such as R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone). The response duration is short and central nervous system relapses are frequent. Whether autologous stem cell transplantation could benefit fit patients responding to CIT remains to be studied.https://www.mdpi.com/2673-6357/3/4/44diffuse large B-cell lymphomaaggressive lymphomahistological transformationIgM lymphoplasmacytic lymphoma<i>MYD88<sup>L265P</sup></i> mutation |
| spellingShingle | Eric Durot Cécile Tomowiak Elise Toussaint Pierre Morel Dipti Talaulikar Prashant Kapoor Jorge J. Castillo Alain Delmer Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment Hemato diffuse large B-cell lymphoma aggressive lymphoma histological transformation IgM lymphoplasmacytic lymphoma <i>MYD88<sup>L265P</sup></i> mutation |
| title | Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment |
| title_full | Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment |
| title_fullStr | Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment |
| title_full_unstemmed | Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment |
| title_short | Transformed Waldenström Macroglobulinemia: Update on Diagnosis, Prognosis and Treatment |
| title_sort | transformed waldenstrom macroglobulinemia update on diagnosis prognosis and treatment |
| topic | diffuse large B-cell lymphoma aggressive lymphoma histological transformation IgM lymphoplasmacytic lymphoma <i>MYD88<sup>L265P</sup></i> mutation |
| url | https://www.mdpi.com/2673-6357/3/4/44 |
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