[Successful treatment of Bing-Neel syndrome using combination therapy with fludarabine and rituximab].
Bing-Neel syndrome is known as Waldenström's macroglobulinemia with central nervous system infiltration by neoplastic lymphoplasmacytoid and plasma cells. A 74-year-old man was admitted because of progressive cognitive impairment. Serum immunoelectrophoresis showed a monoclonal IgM-kappa component. Bone marrow aspiration revealed 59% small lymphocytes showing plasmacytoid differentiation. Bone marrow flow cytometry disclosed a population of kappa light-chain positive lymphoid cells expressing CD19, CD20, CD38, and CD138. Magnetic resonance imaging of the brain demonstrated gadolinium-enhancement in the right temporo-parieto-occipital meninges with sulcal enhancement. Cerebrospinal fluid cytology showed a population of lymphoplasmacytoid cells, positive for CD19, CD20, CD25, and kappa light-chain. Based on these findings, Bing-Neel syndrome was diagnosed. Although combination chemotherapy consisting of intrathecal methotrexate and oral cyclophosphamide was started, his symptoms continued to worsen. Then, we initiated treatment with a regimen consisting of fludarabine/rituximab (FR). After 6 courses of this FR regimen, a complete remission was achieved. Our case suggests the FR regimen to potentially be an effective treatment option for Bing-Neel syndrome of the scattered type.
Department of Hematology and Oncology, Mie University Graduate School of Medicine.
Keiki
K
Nagaharu
Kana
K
Miyazami
Hiroshi
H
Imai
Asako
A
Tamura
Akira
A
Umino
Atsushi
A
Fujieda
Yuka
Y
Sugimoto
Motoko
M
Yamaguchi
Masahiro
M
Masuya
Naoyuki
N
Katayama
D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D001853:Bone Marrow / Q000473:pathology; D004338:Drug Combinations; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D000069283:Rituximab; D014740:Vidarabine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D008258:Waldenstrom Macroglobulinemia / Q000188:drug therapy* / Q000473:pathology
D058846:Antibodies, Monoclonal, Murine-Derived; D004338:Drug Combinations; D000069283:Rituximab; D014740:Vidarabine; C024352:fludarabine