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<p>Waldenstrom macroglobulinemia (WM) is a rare B-cell lympho-proliferative disorder. There is limited data from India regarding the characteristics and outcomes of patients with WM. We describe here the patients with WM treated at our center over the course of the past 15 years. This was a single center, retrospective analysis. Patients fulfilling the diagnosis of WM who were treated at our center between 2009 and 2023 were included in the study. Fifty-five patients were included in the study for analysis. The median age was 62 years with a Male: Female ratio of 3.2:1. Most patients (<i>n</i> = 42; 76.4%) had symptoms attributable to WM, while 5 patients (9.1%) had Immunoglobulin M (IgM) related symptoms and 8 patients (14.5%) had both. The median hemoglobin of the cohort was 7.4&#xa0;g/dl and the median serum IgM level was 4.87&#xa0;g/L. <i>MYD88</i> mutation testing was done in 30 patients and was positive in 16 (53.3%). The most common first line therapy was chemo-immunotherapy (<i>n</i> = 36; 65.5%). Most common chemo-immunotherapy was Bendamustine-Rituximab followed by Rituximab-Cyclophosphamide-Dexamethasone. The overall response rate and major response rate to front-line therapy was 78.2% and 69.1% respectively. The median follow-up for the entire cohort was 30 months. Fourteen patients died during follow-up with the most common cause being progressive disease (<i>n</i> = 6; 42.9%). The median Event-free Survival, Time to Next therapy and Overall Survival were 45 months, 51 months and 150 months respectively. In our WM case series from India, we found a low incidence of <i>MYD88</i> mutations with comparable treatment outcomes to western literature.</p>

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Clinical Characteristics and Treatment Outcomes of Patients with Waldenstrom Macroglobulinemia

  • 期刊论文

  • 发表日期：

    2025年3月29日

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  • Gaurav Prakash,
  • Charanpreet Singh,
  • Pradeep Reddy,
  • K. S. Lekshmon,
  • Arihant Jain,
  • Alka Khadwal,
  • Pulkit Rastogi,
  • Nabhajit Mallik,
  • Amanjit Bal,
  • Pankaj Malhotra

摘要

Waldenstrom macroglobulinemia (WM) is a rare B-cell lympho-proliferative disorder. There is limited data from India regarding the characteristics and outcomes of patients with WM. We describe here the patients with WM treated at our center over the course of the past 15 years. This was a single center, retrospective analysis. Patients fulfilling the diagnosis of WM who were treated at our center between 2009 and 2023 were included in the study. Fifty-five patients were included in the study for analysis. The median age was 62 years with a Male: Female ratio of 3.2:1. Most patients (n = 42; 76.4%) had symptoms attributable to WM, while 5 patients (9.1%) had Immunoglobulin M (IgM) related symptoms and 8 patients (14.5%) had both. The median hemoglobin of the cohort was 7.4 g/dl and the median serum IgM level was 4.87 g/L. MYD88 mutation testing was done in 30 patients and was positive in 16 (53.3%). The most common first line therapy was chemo-immunotherapy (n = 36; 65.5%). Most common chemo-immunotherapy was Bendamustine-Rituximab followed by Rituximab-Cyclophosphamide-Dexamethasone. The overall response rate and major response rate to front-line therapy was 78.2% and 69.1% respectively. The median follow-up for the entire cohort was 30 months. Fourteen patients died during follow-up with the most common cause being progressive disease (n = 6; 42.9%). The median Event-free Survival, Time to Next therapy and Overall Survival were 45 months, 51 months and 150 months respectively. In our WM case series from India, we found a low incidence of MYD88 mutations with comparable treatment outcomes to western literature.