Ann Lab Med 2020; 40(3): 193-200  https://doi.org/10.3343/alm.2020.40.3.193
Clinical, Laboratory, and Bone Marrow Findings of 31 Patients With Waldenström Macroglobulinemia
Ari Ahn, M.D.1, Chan-Jeoung Park, M.D., Ph.D.2, Young-Uk Cho, M.D., Ph.D.2, Seongsoo Jang, M.D., Ph.D.2, Eul-Ju Seo, M.D., Ph.D.2, Jung-Hee Lee, M.D., Ph.D.3, Dok Hyun Yoon, M.D., Ph.D.3, and Cheolwon Suh, M.D., Ph.D.3
1Department of Laboratory Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea; 2Departments of Laboratory Medicine and 3Internal Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea
Corresponding author: Chan-Jeoung Park, M.D., Ph.D.
Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea
Tel: +82-2-3010-4508 Fax: +82-2-478-0884 E-mail: cjpark@amc.seoul.kr
Received: April 2, 2019; Revised: May 29, 2019; Accepted: October 24, 2019; Published online: May 1, 2020.
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Abstract
Background: Waldenström macroglobulinemia (WM) is a subset of lymphoplasmacytic lymphoma (LPL) with bone marrow (BM) involvement and an IgM monoclonal gammopathy of any level. We aimed to identify the clinical, laboratory, and BM findings of patients with WM and to evaluate the usefulness of CD154 for the diagnosis and prognosis of WM.
Methods: We reviewed the medical records and BM studies and/or flow cytometric immunotyping of 31 patients with untreated WM. Semiquantitative immunohistochemistry (CD20, CD138, tryptase, and CD154) of BM was performed.
Results: Only six patients presented with symptoms of hyperviscosity syndrome. Eleven patients had solid cancer and/or another hematologic malignancy. Mast cells (MC) increased in all samples, with some in close contact with tumor cells. Tryptase-positive MC (17.1/ high-power fields [HPF], 1.2–72.0/HPF) and CD154-positive MC (8.6/HPF, 0.1–31.1/HPF) were observed. The high CD154-positive MC (≥8.6/HPF) group showed a lower overall five-year survival rate than the low CD154-positive MC (<8.6/HPF) group (71.9% vs. 100.0%; P=0.012). Flow cytometric immunophenotyping of BM aspirates showed increased B lymphocytes and plasma cells with a normal phenotype (CD138+/CD38+/CD19+/CD45+/CD56).
Conclusions: Approximately one third of WM patients showed other malignancies and all patients had increased MC. Immunohistochemistry and flow cytometric immunophenotyping are useful for diagnosing WM, and increased CD154-positive MC can indicate poor prognosis.
Keywords: Waldenström macroglobulinemia, Monoclonal gammopathy, Mast cell, CD154



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