Clinical Features of Waldenstrom Macroglobulinemia in Korea.
- Author:
Soo Mee BANG
1
;
Sook Ryun PARK
;
Se Hoon PARK
;
Eun Kyung CHO
;
Sung Soo YOON
;
Dong Bok SHIN
;
Jae Hoon LEE
;
Seonyang PARK
;
Byoung Kook KIM
;
Noe Kyeong KIM
Author Information
1. Department of Internal Medicine, Gachon Medical School, Incheon, Korea.
- Publication Type:Original Article
- Keywords:
Waldenstrom macroglobulinemia;
Lymphoproliferative disorder;
IgM
- MeSH:
Adult;
Aged;
Aged, 80 and over;
Female;
Humans;
Immunoglobulin M/blood;
Korea;
Male;
Middle Aged;
Retrospective Studies;
Waldenstrom Macroglobulinemia/blood/*diagnosis
- From:The Korean Journal of Internal Medicine
2004;19(3):137-140
- CountryRepublic of Korea
- Language:English
-
Abstract:
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/micro L, and 138, 000/micro L, 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.
