WALDENSTROM MACROGLOBULINEMIA

Waldenstrom Macroglobulinemia

Abstract

Waldenstrom Macroglobulinemia is a low-grade B-cell lymphoma distinuished by monoclonal synthesis and secretion of IgM antibodies. The classic symptoms of WM outcome from blood hyper viscosity. Neurologic symptoms, for example fatigue, dizziness, and distorted dream, are common. The metastatic disperse to the CNS, which seldom has been described, can occur either diffusely or in the pattern of a mass lesion.



Waldenstrom Macroglobulinemia

Study Design

A study was assumed to evaluate the frequency and natural history of syndrome in patients with asymptomatic Waldenstrom Macroglobulinemia (WM). Among 132 consecutive, newly diagnosed patients with monoclonal IgM, 82 (27%) had symptomatic WM designated by anemia, lymphadenopathy, or splenomegaly. Thirty-one patients had similar clinical features but were asymptomatic and followed without therapy until disease progression. There were 19 patients with monoclonal gammopathy of undetermined significance of IgM type (MGUS). In comparison to overt WM, patients with asymptomatic WM had significantly higher hemoglobin (Hgb) level (median, 12.1 v 9.7 g/dL), lower serum ß2-microglobulin (B2M) level (median, 2.4 v 3.4 mg/L), and similar IgM peaks (median, 2.2 and 1.8 g/dL). The IgM element was 3.6 g/dL or less in all patients with asymptomatic disease. For asymptomatic WM, median time to disease progression was 6.9 years with rare morbidity. Prognostic factors for early progression were Hgb <11.5 g/dL, B2M = 3.0 mg/L, and IgM peak >3.0 g/dL. Combinations of these variables defined three risk groups for progression with markedly different median times to progression of >5 years, 2 years, and 0.5 year, respectively. Response rate and survival after institution of treatment were similar to those of patients treated promptly for overt disease (Linet, 1993).

Introduction

Waldenstrom Macroglobulinemia (WM) is a lymphoproliferative disorder described by the presence of monoclonal lymphocytes that produce monoclonal immunoglobulin M (IgM). This disease was originally described in 1944 by Jan Waldenström, who reported two patients with long-standing oronasal bleeding, mild generalized lymphadenopathy, important anemia, elevated erythrocyte sedimentation rate, very high serum viscosity, and low levels of fibrinogen. Large amounts of a high-molecular-weight globulin were detected in the serum of these patients, and their bone marrow aspirate revealed increased numbers of lymphocytoid cells. The abnormal serum globulin was subsequently recognized as an immunoglobulin and was called immunoglobulin M. Under the diagnosis of Waldenstrom's macroglobulinemia are included patients with a low-grade lymphoplasmatic disorder associated with various amounts of serum monoclonal IgM. Some individuals, however, have a monoclonal IgM of undetermined significance without symptoms, without organomegaly or lymphadenopathy, and without anemia or evidence of bone marrow infiltration by the lymphoma. Such individuals do not require treatment, but some may develop overt WM (Owen, 2001).

Waldenstrom Macroglobulinemia is an unusual disease. Herrinton and Weiss reported an age-standardized annual incidence rate of 6.1 cases/million in white men and 2.5/million in white women. This disease is much more common in whites than in blacks. Groves et al found that the age-adjusted incidence rates for WM/1 million person-years at risk were 3.4 among men and 1.7 among women. The rates increased sharply with age, from 0.1 at ages under 45 years to ...