Clinical features of Waldenstrom macroglobulinemia in Korea
| Autor: | Sook Ryun Park, Jae-Hoon Lee, Eun Kyung Cho, Sung-Soo Yoon, Dong Bok Shin, Seon Yang Park, Soo Mee Bang, Byoung Kook Kim, Se Hoon Park, Noe Kyeong Kim |
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| Rok vydání: | 2004 |
| Předmět: |
Adult
Male medicine.medical_specialty IgM medicine.medical_treatment Gastroenterology Internal medicine medicine Humans Aged Retrospective Studies Aged 80 and over Chemotherapy Korea biology business.industry Waldenstrom macroglobulinemia Retrospective cohort study Middle Aged medicine.disease Surgery Peripheral neuropathy Transitional cell carcinoma B symptoms Immunoglobulin M Lymphoproliferative disorder biology.protein Plasmapheresis Female Original Article medicine.symptom business |
| Zdroj: | The Korean Journal of Internal Medicine |
| ISSN: | 1226-3303 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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