Low-grade B-cell lymphoma presenting primarily in the bone marrow

• Published in: Human pathology 2014-07, Vol.45 (7), p.1379-1387
• Main Authors: Iwatani, Kayoko, MD, Takata, Katsuyoshi, MD, PhD, Sato, Yasuharu, MD, PhD, Miyata-Takata, Tomoko, MD, PhD, Iwaki, Noriko, MD, Cui, Wei, PhD, Sawada-Kitamura, Seiko, MD, PhD, Sonobe, Hiroshi, MD, PhD, Tamura, Maiko, MD, PhD, Saito, Katsuhiko, MD, PhD, Miyatani, Katsuya, MD, PhD, Yamasaki, Rie, MD, PhD, Yamadori, Ichiro, MD, PhD, Fujii, Nobuharu, MD, PhD, Terasaki, Yasushi, MD, Maeda, Yoshinobu, MD, PhD, Tanimoto, Mitsune, MD, PhD, Nakamura, Naoya, MD, PhD, Yoshino, Tadashi, MD, PhD
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Bone marrow; Bone Marrow - pathology; Classification; Female; Flow cytometry; Humans; Leukemia; LGBCL-NOS; Low-grade B cell lymphoma; Lymphoma; Lymphoma, B-Cell - diagnosis; Lymphoma, B-Cell - genetics; Lymphoma, B-Cell - pathology; Male; Middle Aged; Mutation; MYD88; Neoplasm Grading; Paraproteinemias - diagnosis; Paraproteinemias - genetics; Paraproteinemias - pathology; Pathology; Patients; Retrospective Studies; Tomography
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2014.02.010

Summary Cases of low-grade B-cell lymphoma presenting primarily in the bone marrow are rare, and its clinicopathology remains unclear. We retrospectively examined patients with low-grade B-cell lymphoma presenting primarily in the bone marrow. Fourteen patients met the inclusion criteria, including 5 with lymphoplasmacytic lymphoma (LPL), 3 with chronic lymphocytic leukemia/small lymphocytic lymphoma, 2 with follicular lymphoma (FL), and 4 with low-grade B-cell lymphoma not otherwise specified (LGBCL-NOS). The median age was 69.5 years (range, 42-89 years), and a slight male predominance was noted (9 men and 5 women, 1.8: 1). Immunohistochemically, all cases were positive for CD20. One case was positive for CD138. Both cases of FL were positive for CD10 and B-cell lymphoma 2 (BCL-2), and immunoglobulin heavy locus ( IgH )/ B-cell lymphoma 2 rearrangement was observed by fluorescence in situ hybridization. The myeloid differentiation primary response gene ( 88 ) leucine to proline mutation was observed in 3 of 5 LPL, 1 of 2 FL, and 2 of 4 LGBCL-NOS patients. Paraproteinemia was observed in 10 patients; IgM and IgG paraproteinemia were observed in 6 and 3 patients, respectively. In this patient series, 3 patients had died at a median follow-up of 36.5 months; the cause of death of 1 LPL patient was malignant lymphoma itself. Thus, low-grade B-cell lymphoma presenting primarily in the bone marrow has various subtypes, and approximately one-third of the patients had LGBCL-NOS. The immunophenotypic features and myeloid differentiation primary response gene ( 88 ) leucine to proline mutation data of LGBCL-NOS suggested that some cases present with characteristics similar to those of LPL or marginal zone lymphoma.