Treatment strategy in a patient showing borderline features between plasmablastic lymphoma and plasmablastic myeloma har
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LETTER TO THE EDITOR
Treatment strategy in a patient showing borderline features between plasmablastic lymphoma and plasmablastic myeloma harboring a 17p deletion Kyoko Yoshihara 1 & Satoshi Yoshihara 1,2 & Ikuo Matsuda 3 & Takehito Imado 4 & Shohei Matsuo 5 & Masaya Okada 1 & Yoshifumi Shimizu 4 & Seiichi Hirota 3 & Yoshihiro Fujimori 1,2 Received: 8 February 2020 / Accepted: 24 February 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Dear Editor, Plasmablastic lymphoma (PBL) is a highly aggressive lymphoma with a diffuse proliferation of CD20-negative large neoplastic cells, resembling B immunoblasts or plasmablasts [1]. Although it is known to be associated with human immunodeficiency virus (HIV) infection, cases in immunocompetent patients have been increasingly reported. Plasmablastic myeloma (PBM) is an aggressive form of multiple myeloma with a proliferation of plasmablasts in the bone marrow as well as extramedullary lesion. These two disease entities share common features including expression of plasma cell markers CD138, CD38, and MUM-1 and lack of expression of B cell markers CD20 and PAX-5. Thus, differential diagnosis between PBL and PBM is often difficult [2–4]. A 42-year-old woman underwent incisional biopsy for a breast tumor. Diffuse proliferation of large atypical lymphoid cells with prominent nucleoli was observed (Fig. 1a). The tumor cells were positive for CD138, CD79a, MUM-1, and c-myc and negative for CD20 and Epstein-Barr virus– encoded small RNA (EBER) (Fig. 1b, c). The Ki-67 labeling index was > 90% (Fig. 1c). Computed tomography (CT) and
* Satoshi Yoshihara [email protected] 1
Division of Hematology, Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
2
Department of Transfusion Medicine and Cellular Therapy, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
3
Department of Surgical Pathology, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
4
Department of Hematology, Takarazuka City Hospital, Takarazuka, Hyogo, Japan
5
Department of Pathology, Takarazuka City Hospital, Takarazuka, Hyogo, Japan
positron emission tomography (PET)/CT revealed multiple bulky tumors (up to 13 cm) in the breast, liver, pelvis, and uterus and multiple bone lesions (Fig. 1e, g). Fluorescent in situ hybridization analysis of the tumor cells showed the presence of a 17p deletion. Blood test showed an elevated serum free light chain ratio (kappa 1920 mg/L, lambda 17.3 mg/L); urine test showed BJP-kappa type proteinuria (4.2 g/day). Repeated bone marrow aspirations showed the complete absence of abnormal plasma cells, confirmed via 8-color flow cytometry. The presence of multiple extramedullary/ extranodal tumors in the absence of bone marrow involvement and myeloma-defining events (except bone lesions) supported the PBL diagnosis. However, the presence of BJ-type proteinuria and bone lesions favored the PBM diagnosis. Based on the borderline features of PBL and PBM, we planned a two-phase strategy. First, to debulk the bulky tumors via in
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