Definition

Waldenstrom macroglobulinemia (WM) is a distinct B-cell disorder resulting from the accumulation, predominantly in the bone marrow, of clonally related lymphoplasmacytic cells, which secrete a monoclonal IgM protein. The paramters predicting prognosis of WM are list below:

Table 1 International Prognostic Scoring System for WM.

Factor associated with WM prognosisValue
Age, yr>65
Hemoglobin, g/dL≤11.5
Platelet count, No./mcL≤100,000
β2-Microglobulin, mg/L>3
Monoclonal IgM, g/dL>7

Risk stratum and survival

Risk categoryScoreMedian survival, mon
Low0 or 1 (except age)142.5
Intermediate2 or age >65 yr98.6
High>243.5

What can WM result in?

  1. Hyperviscosity syndrome. The symptoms of hyperviscosity are due primarily to shear forces that rupture unsupported venous channels. The presentation generally includes epistaxis, gingival bleeding, and visual changes due to retinal hemorrhages.
  2. Peripheral neuropathy. Autoantibody activity to myelin-associated glycoprotein, ganglioside M1, sulfatide moieties on peripheral nerve sheaths can result in peripheral neuropathy.
  3. Hemolytic anemia. Cold or warm antibodies in WM patients can result in autoimmune hemolytic anemia.
  4. Immune complex vasculitis.

Management of WM

The identification of the asymptomatic patient is important because this group of patients do not need therapy. What this group need is closely observation. For patients with symptoms of WM, therapy should be initiated. Frontline treatment options for WM include oral alkylators, nucleoside analogs, rituximab, or the combinations of these three. Other durgs to be considered includes bortezomib and thalidomide. The goal is to reduce the paraprotein level. (more…)