Population-based study on the impact of the familial form of Waldenström macroglobulinemia on overall survival

Steingrímsson, Vilhjálmur; Lund, Sigrún H.; Turesson, Ingemar; Goldin, Lynn R.; Björkholm, Magnus; Landgren, Ola; Kristinsson, Sigurður Y.

doi:10.1182/blood-2015-01-622068

Cited by 21 publications

(12 citation statements)

References 7 publications

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“… 69 The diagnosis of familial form represents an independent marker for disease progression being associated with a 1.3-fold increased risk of death compared to sporadic WM, with an increasing hazard ratio for each additional relative with a lymphoproliferative disorder (defined as WM, NHL, MM, CLL, or MGUS). 70 From a clinical point of view, greater BM involvement and baseline IgM level were observed in familial compared to sporadic WM, while no difference was noted in cytogenetic abnormalities or lymph node or spleen involvement. 71 …”

Section: Prognosismentioning

confidence: 94%

Waldenstrom’s Macroglobulinemia: An Update

Mazzucchelli

Frustaci

Deodato

et al. 2018

Mediterr J Hematol Infect Dis

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Waldenstrom Macroglobulinemia is a rare lymphoproliferative disorder with distinctive clinical features.Diagnostic and prognostic characterisation in WM significantly changed with the discovery of two molecular markers: MYD88 and CXCR4. Mutational status of these latter influences both clinical presentation and prognosis and demonstrated therapeutic implications.Treatment choice in Waldenstrom disease is strictly guided by patients age and characteristics, specific goals of therapy, the necessity for rapid disease control, the risk of treatment-related neuropathy, disease features, the risk of immunosuppression or secondary malignancies and potential for future autologous stem cell transplantation.The therapeutic landscape has expanded during the last years and the approval of ibrutinib, the first drug approved for Waldenstrom Macroglobulinemia, represents a significant step forward for a better management of the disease.

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Section: Prognosismentioning

confidence: 94%

Waldenstrom’s Macroglobulinemia: An Update

Mazzucchelli

Frustaci

Deodato

et al. 2018

Mediterr J Hematol Infect Dis

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“…Incidence is higher in whites (4.1 per million per year) than in blacks (1.8 per million per year) . Waldenström patients had a positive family history of lymphoplasmacytic lymphoma or WM in 4.3%, and a family history was associated with poorer survival than the nonfamilial forms . A study of monoclonal immunoglobulins showed that the M protein isotype in black and white patients was 2% and 16% IgM, respectively.…”

Section: Disease Overviewmentioning

confidence: 99%

Waldenström macroglobulinemia: 2019 update on diagnosis, risk stratification, and management

Gertz

2018

American J Hematol

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Disease Overview Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity. Diagnosis Presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in >90% of patients and is found in the majority of IgM monoclonal gammopathy of undetermined significance patients. Risk Stratification Age, hemoglobin level, platelet count, β2 microglobulin, and monoclonal IgM concentrations are characteristics that are predictive of outcomes. Risk‐Adapted Therapy Not all patients who fulfill WM criteria require therapy; these patients can be observed until symptoms develop. Rituximab‐monotherapy is inferior to regimens that combine it with bendamustine, an alkylating agent, a proteosome inhibitor, or ibrutinib. Purine nucleoside analogs are active but usage is declining for less toxic alternatives. The preferred Mayo Clinic induction is rituximab and bendamustine. Potential for stem cell transplantation should be considered in selected younger patients. Management of Refractory Disease Bortezomib, fludarabine, thalidomide, everolimus, ibrutinib, carfilzomib, lenalidomide, and bendamustine have all been shown to have activity in relapsed WM. Given WM's natural history, reduction of therapy toxicity is an important part of treatment selection.

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“…Familial WM has been correlated to an younger age and a higher BM involvement at the time of diagnosis 19 . In addition, familial disease has an increased risk of death (HR 1.3) compared to sporadic disease 20 , and it was shown to be an independent risk factor for disease progression (HR 0.554) 21 . Patients with familial WM have inferior treatment responses, shorter time to progression, and shorter time to next therapy with rituximab therapy compared to sporadic WM; although they have improved outcomes with bortezomib-containing regimens 21 .…”

Section: Pathogenesismentioning

confidence: 99%

Waldenström Macroglobulinemia: Review of Pathogenesis and Management

Yun

Johnson

Okolo

et al. 2017

Clinical Lymphoma Myeloma and Leukemia

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Waldenström macroglobulinemia (WM) is a low-grade B-cell clonal disorder characterized by lymphoplasmacytic bone marrow involvement associated with monoclonal immunoglobulin M (IgM). Although WM remains to be an incurable disease with a heterogeneous clinical course, the recent discovery of mutations in the MYD88 and CXCR4 genes further enhanced our understanding of its pathogenesis. Development of new therapies including monoclonal antibodies, proteasome inhibitors, and Bruton’s tyrosine kinase inhibitors have made the management of WM increasingly complex. Treatment should be tailored to the individual patient while considering many clinical factors. The clinical outcomes are expected to continue to improve given the emergence of novel therapeutics and better understanding of the underlying pathogenesis.

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Population-based study on the impact of the familial form of Waldenström macroglobulinemia on overall survival

Cited by 21 publications

References 7 publications

Waldenstrom’s Macroglobulinemia: An Update

Waldenstrom’s Macroglobulinemia: An Update

Waldenström macroglobulinemia: 2019 update on diagnosis, risk stratification, and management

Waldenström Macroglobulinemia: Review of Pathogenesis and Management

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