Prognostic factors and response to fludarabine therapy in patients with Waldenström macroglobulinemia: results of United States intergroup trial (Southwest Oncology Group S9003)

Diagnosis and Management of Waldenström Macroglobulinemia: Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines

“…Subsequent studies have evaluated the role of purine nucleoside analogues and found that this class of agents is effective in Waldenström macroglobulinemia. [50][51][52][53][54] Because alkylating agents and nucleoside analogues have been shown to be effective, combinations of these agents have been tested, and response rates of 58% to 88% have been observed. [55][56][57] The addition of rituximab to these agents has been well tolerated with little additional toxicity and appears to further increase the response rate.…”

Clinical characteristics and factors predicting evolution of asymptomatic IgM monoclonal gammopathies and IgM-related disorders

“…The unequivocal histopathological evidence of bone marrow lymphoplasmacytic (LP) lymphoma (LP-NHL) irrespective of IgM concentration has been suggested to distinguish smouldering WM (sWM) from IgM-MGUS. [2][3][4][5][6][7][8][9]11,17,18 Whether this criterion allows the identification of two different prognostic subgroups has not been formally proved yet.…”

“…2,3,6,9 Although the majority will not develop symptoms, subsets of patients evolve to overt lymphoid malignancy with variable frequency. [2][3][4][5][12][13][14][15][16][17] Studies defining factors predicting malignant transformation in aIgM-MGs are lacking, and no criterion exists reliably distinguishing subpopulations with different evolution probability to active disease. The unequivocal histopathological evidence of bone marrow lymphoplasmacytic (LP) lymphoma (LP-NHL) irrespective of IgM concentration has been suggested to distinguish smouldering WM (sWM) from IgM-MGUS.…”

“…It includes MG of undetermined significance (MGUS) and preclinical WM. [1][2][3][4][5][6][7][8][9][10][11] In the absence of active lymphoproliferative disease, aIgM-MGs ought to be observed without treatment. 2,3,6,9 Although the majority will not develop symptoms, subsets of patients evolve to overt lymphoid malignancy with variable frequency.…”

“…[1][2][3][4][5][6][7][8][9][10][11] In the absence of active lymphoproliferative disease, aIgM-MGs ought to be observed without treatment. 2,3,6,9 Although the majority will not develop symptoms, subsets of patients evolve to overt lymphoid malignancy with variable frequency. [2][3][4][5][12][13][14][15][16][17] Studies defining factors predicting malignant transformation in aIgM-MGs are lacking, and no criterion exists reliably distinguishing subpopulations with different evolution probability to active disease.…”