Ann Lab Med.  2020 May;40(3):193-200. 10.3343/alm.2020.40.3.193.

Clinical, Laboratory, and Bone Marrow Findings of 31 Patients With Waldenström Macroglobulinemia

• Ahn A
• Park CJ
• Cho YU
• Jang S
• Seo EJ
• Lee JH
• Yoon DH
• Suh C

• KMID: 2466011
• DOI: http://doi.org/10.3343/alm.2020.40.3.193

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.

Keyword

Waldenström macroglobulinemia; Monoclonal gammopathy; Mast cell; CD154

MeSH Terms

B-Lymphocytes
Bone Marrow*
Diagnosis
Hematologic Neoplasms
Humans
Immunoglobulin M
Immunohistochemistry
Immunophenotyping
Lymphoma
Mast Cells
Medical Records
Paraproteinemias
Phenotype
Plasma Cells
Prognosis
Survival Rate
Tryptases
Waldenstrom Macroglobulinemia*
Immunoglobulin M
Tryptases

Figure

• 

  Fig. 1 Clinical and laboratory findings of 31 patients with Waldenström macroglobulinemia. Y-axis indicates the percentage of patients with those findings.

• 

  Fig. 2 BM biopsy findings of patients with WM. (A) Classical lymphoplasmacytic lymphoma in a patient with WM (H&E stain, ×200 and ×400). (B) Representative immunohistochemistry for CD20 and CD138 (×400). (C) Representative immunohistochemistry for kappa and lambda light chains (×400). (D) Immunohistochemistry for tryptase and CD154 (×400) highlights increased mast cells (arrow).Abbreviations: BM, bone marrow; WM, Waldenström macroglobulinemia; H&E, hematoxylin and eosin.

• 

  Fig. 3 Overall survival curves for the high and low CD154-positive mast cell groups based on the median value of 8.6/HPF.Abbreviation: HPF, high-power field.

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