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Waldenström's macroglobulinemia

Background

Overview

Definition
Waldenstrom's macroglobulinemia is a type of lymphoplasmacytic lymphoma associated with monoclonal IgM gammopathy.
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Pathophysiology
Waldenstrom's macroglobulinemia is caused by an uncontrolled clonal proliferation of terminally differentiated B lymphocytes, which may be due to sporadic or inherited cytogenetic abnormalities.
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Epidemiology
The estimated incidence of Waldenstrom's macroglobulinemia in the US is 0.57 per 100,000 person-years.
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Disease course
Production of IgM paraproteins by Waldenstrom's macroglobulinemia clones leads to clinical manifestations of peripheral neuropathy, systemic amyloidosis, hyperviscosity syndrome, congestive HF, cryoglobulinemia, and death.
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Prognosis and risk of recurrence
The 5-year survival rates for patients with low risk, intermediate risk, and high-risk patients Waldenstrom's macroglobulinemia are 87%, 68%, and 36%, respectively. Treatment-related 1-year mortality rates of up to 44% limit the use of allogeneic transplantation.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of Waldenstrom's macroglobulinemia are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2022,2014), the European Society of Medical Oncology (ESMO 2018), and the International Workshop on Waldenstrom's Macroglobulinemia (IWWM 2006). ...
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Screening and diagnosis

Diagnostic criteria: as per ESMO 2018 guidelines, diagnose WM in patients who meet both of the following criteria:
serum protein electrophoresis showing a monoclonal IgM of any amount, confirmed by immunofixation
bone marrow biopsy showing infiltration by monoclonal lymphoplasmacytic cells.
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  • Screening of family relatives

  • Differential diagnosis

Classification and risk stratification

Risk assessment: as per BSH 2022 guidelines, insufficient evidence to support the use of IPSS for WM or other prognostic scoring systems in determining treatment approaches for individual patients, whilst potentially useful to guide discussions with patients.
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International Prognostic Scoring System (IPSS) for Waldenstrom's macroglobulinemia
Age > 65 years
Hemoglobin ≤ 11.5 g/dL
Platelet count ≤ 100×10⁹/L
Serum beta2-microglobulin > 3 mg/L
Monoclonal IgM concentration > 70 g/L
5-year survival is 87%

Diagnostic investigations

Laboratory evaluation: as per BSH 2022 guidelines, obtain sequential monitoring of IgM/monoclonal protein in a single laboratory using a single methodology.
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  • Screening for viral hepatitis and HIV

  • Diagnostic imaging

  • Evaluation for complications

Diagnostic procedures

Bone marrow biopsy: as per BSH 2022 guidelines, perform bone marrow aspiration and trephine biopsy for definitive diagnosis of WM as well as in all patients with suspected symptomatic WM or other IgM-disorder.
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  • Tissue biopsy

  • Cytogenetic analysis

Medical management

Indications for treatment: as per ESMO 2018 guidelines, initiate treatment in patients with WM who develop B symptoms, cytopenias, hyperviscosity, moderate or severe neuropathy, amyloidosis, symptomatic cryoglobulinemia or cold agglutinin disease.
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  • Multidisciplinary care

  • First-line therapy

  • Second-line therapy, frail/unfit patients

  • Maintenance therapy

  • Management of relapsed disease

  • Management of refractory disease

Therapeutic procedures

Stem cell transplantation: as per BSH 2022 guidelines, consider offering ASCT as a second or later line of therapy in selected chemotherapy-responsive patients with a relapse.
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  • Plasmapheresis

Specific circumstances

Patients with histological transformation
As per BSH 2022 guidelines:
Treat high-grade transformation to diffuse large B-cell lymphoma with similar regimens used to treat de novo diffuse large B-cell lymphoma, but prognosis is less favorable.
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Consider offering ASCT as consolidation for high-grade transformation in suitable patients responding to induction chemotherapy.
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  • Patients with neuropathy

  • Patients with cold agglutinin disease

  • Patients with cryoglobulinemia

  • Patients with hypogammaglobulinemia

  • Patients with anemia

  • Patients with amyloidosis

Preventative measures

Routine immunizations: as per BSH 2022 guidelines, offer pneumococcal vaccination in the form of pneumococcal conjugate vaccine followed by pneumococcal polysaccharide vaccine at least 2 months later in all patients with WM.
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  • Pneumocystis and herpes prophylaxis

Follow-up and surveillance

Assessment of treatment response: as per BSH 2022 guidelines, use uniform criteria for the assessment of treatment response.
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