Waldenström's macroglobulinaemia (WM), (previously also known as lymphoplasmacytic lymphoma (LPL)), is a type of B-cell lymphoma. It is a rare condition, accounting for only 1% of all lymphoproliferative disorders.
Recent publications classify Waldenström's macroglobulinaemia as an lymphoplasmacytic lymphoma with any level of an IgM paraprotein 10. Therefore the two entities are not synonymous.
The incidence is 1,500 cases per annum in the United States. The median age of onset is 60-65 years. A minority of cases occur in the late teens.
Most patients are asymptomatic at time of diagnosis, which is often incidentally made during blood tests for an unrelated condition. If symptoms occur, the most common ones are fatigue (2/3 of cases), and polyneuropathy (1/4 of cases). Otherwise, presentation includes the triad of non-specific B-symptoms: fever, night sweats and unintentional weight loss. Another feature is hyperviscosity syndrome.
The pathogenesis is poorly understood, but may resemble that of CLL. A clonal proliferation of functionally impaired B-cells occurs, as well as infiltration of various tissues (predominantly bone marrow, spleen and lymph nodes). There is overproduction of IgM, leading to hyperviscosity syndrome and resultant impaired microcirculation.
- Bing-Neel syndrome: malignant lymphocyte infiltration into the central nervous system (CNS) 11
Treatment and prognosis
The International Prognostic Scoring System for Waldenström's macroglobulinaemia is a predictive model for characterisation of long-term outcome. Factors predicting survival are:
- age >65 years
- haemoglobin ≤11.5 g/dL
- platelet count ≤100×109/L
- B2-microglobulin >3 mg/L
- serum monoclonal protein concentration >70 g/L
Risk categories are:
- low: ≤1 adverse variable except age
- intermediate: 2 adverse characteristics or age >65 years
- high: >2 adverse characteristics
Five-year survival rates for these categories are 87%, 68% and 36% respectively.
Untreated, the disease typically progresses indolently, and behaves differently among patients.
History and etymology
This condition was first described in 1944 by Swedish oncologist Jan G. Waldenström.
- overview of lymphoma
WHO classification of tumours of haematopoietic and lymphoid tissues
- Hodgkin lymphoma
mature B-cell lymphoma
- Burkitt lymphoma
- follicular lymphoma
- lymphoplasmacytic lymphoma (Waldenström's macroglobulinaemia)
- lymphomatoid granulomatosis
- mantle cell lymphoma
- mature T-cell and NK-cell lymphoma
- post-transplant lymphoproliferative/lymphoproliferation disorders
- mature B-cell lymphoma
- location-specific lymphomas
- central nervous system
- head and neck lymphoma
- thoracic lymphoma
- gastrointestinal lymphoma
- hepatobiliary lymphoma
- genitourinary lymphoma
- musculoskeletal lymphoma
- cutaneous lymphoma
- lymphoma staging
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