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.
Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Immunoglobulin M/blood ; Korea ; Male ; Middle Aged ; Retrospective Studies ; Waldenstrom Macroglobulinemia/blood/*diagnosis

OBJECTIVETo investigate the clinicopathologic features of lymphoplasmacytic lymphoma (LPL) with Waldenström's macroglobulinemia (WM) and to evaluate the usefulness of immunophenotype analysis in diagnosis and differential diagnosis of the tumor.

METHODSA total of 40 cases of LPL with WM diagnosed according to the 2008 World Health Organization classification of tumors of hematopoietic and lymphoid tissues were analyzed using immunophenotype and follow-up information.

RESULTSThe mostly common initial clinical presentations were non-specific symptoms, such as fatigue, anemia and hemorrhage. Lymphadenopathy, splenomegaly and hepatomegaly were found in 42.5%, 20.0% and 12.5% of the patients respectively. The pattern of bone marrow involvement included mixed type (47.2%), diffuse type (41.7%) and interstitial type (11.1%). The nodal architecture was completely destroyed in one case and partially effaced with residual germinal centers and dilated sinuses in 8 cases. All of the neoplastic cells expressed CD20 and CD79a. Neoplastic plasma cells were positive for CD138 and CD79a. No cases expressed CD5. Four cases weakly expressed CD23. No significant prognosis related factors were identified in the survival analysis.

CONCLUSIONSLPL with WM is a rare indolent small B-cell lymphoma, which is commonly seen, in older male patients. The tumor frequently involves bone marrow and shows various clinical manifestations. Combination analyses of the bone marrow biopsy histology, immunophenotypic study and clinical data, especially the serum examination are important for the diagnosis of LPL with WM.

Adult ; Aged ; Aged, 80 and over ; Antigens, CD20 ; metabolism ; Bone Marrow ; metabolism ; pathology ; CD79 Antigens ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Immunoglobulin M ; blood ; Immunophenotyping ; Leukemia, Lymphocytic, Chronic, B-Cell ; metabolism ; pathology ; Lymphatic Metastasis ; Lymphoma, B-Cell, Marginal Zone ; metabolism ; pathology ; Lymphoma, Follicular ; metabolism ; pathology ; Lymphoma, Mantle-Cell ; metabolism ; pathology ; Male ; Middle Aged ; Multiple Myeloma ; metabolism ; pathology ; Neoplasm Invasiveness ; Survival Rate ; Syndecan-1 ; metabolism ; Waldenstrom Macroglobulinemia ; immunology ; metabolism ; pathology