Clinical features of Waldenstrom macroglobulinemia in Korea.

BACKGROUND

Waldenstrom macroglobulinemia (WM) is a lymphoproliferative disorder characterized by monoclonal IgM. Its rarity makes it difficult to know the clinical manifestations and outcomes of patients with WM.

METHODS

The clinical records of 13 patients diagnosed with WM between 1983 and 2003 were reviewed, and 12 patients were eligible.

RESULTS

The median age was 57 years (range, 40 to 85), and the male to female ratio was 2. B symptoms and hyperviscosity requiring plasmapheresis existed in 5 and 4 patients, respectively, at the time of diagnosis. Hepatomegaly and splenomegaly were detected in 5 and 3 patients, respectively. Sites of extranodal involvement were bone (3) and lung (1) in 3 patients. The peripheral neuropathy was complicated in 3 patients. (Ed note: check this sentence.) Cryoglobulin was checked in 6 patients and it was detected in 3 of them. The median concentration of serum IgM was 4.2 g/dL (0.7-6.2). The median albumin, hemoglobin, WBC, and platelet levels were 2.8 g/dL, 8 g/dL, 5,400/microL, and 138,000/microL, respectively. One patient had transitional cell carcinoma concomitantly, and one patient developed small cell lung cancer. Of the 11 patients receiving chemotherapy (7-chlorambucil, 2-melphalan, 1-cyclophosphamide, 1-CHOP), 4 patients showed the objective responses including 2 complete remissions, but they all ultimately relapsed. The response rate of second-line therapy was 14% (1/7). After a median follow-up of 20 months, 3 patients were still alive with disease. The median overall and progression-free survival were 24 months (95% confidence interval (CI): 5-43) and 24 months (95% CI: 8-40), respectively.

CONCLUSION

The initial high levels of serum IgM and severe anemia reflect a lack of suspicion of WM at the early stage. Careful suspicion and proper diagnostic approaches will allow more patients to show an improved outcome.