Lymphomatoid granulomatosis involving central nervous system successfully treated with rituximab alone.
OBJECTIVE
To report the successful treatment of a patient with lymphomatoid granulomatosis (LYG), a rare Epstein-Barr virus-positive lymphoproliferative disorder, using rituximab (anti-CD20 monoclonal antibody). The prognosis for LYG has been reported to be poor, and no satisfactory treatment has been established. Because central nervous system (CNS) involvement of LYG has been known to show poor prognosis, the establishment of an effective treatment for CNS LYG with mild adverse effects is desired.


METHODS
Case report.


METHODS
University hospital.


METHODS
A 48-year-old Japanese man presenting with slowly progressive spastic paraparesis diagnosed as LYG involving the CNS and lungs.


METHODS
The patient was treated with rituximab (375 mg/m2, once weekly for 1 month) alone. Main Outcome Measure Improvement of the lesions on imaging.


RESULTS
The neurological signs resolved and the lesions in the CNS and lungs were mostly diminished after the rituximab monotherapy without any adverse effects. The patient stayed in remission for 18 months.


CONCLUSIONS
Rituximab monotherapy was effective in treating the patient; hence, rituximab should be considered as the initial treatment against LYG involving the CNS.
Ishiura
Hiroyuki
H
Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Morikawa
Masato
M
Hamada
Masashi
M
Watanabe
Takuro
T
Kako
Shinichi
S
Chiba
Shigeru
S
Motokura
Toru
T
Hangaishi
Akira
A
Shibahara
Junji
J
Akahane
Masaaki
M
Goto
Jun
J
Kwak
Shin
S
Kurokawa
Mineo
M
Tsuji
Shoji
S
D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D001921:Brain / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology; D002490:Central Nervous System / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology; D016543:Central Nervous System Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D020031:Epstein-Barr Virus Infections / Q000150:complications; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D008168:Lung / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology; D008175:Lung Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D008214:Lymphocytes / Q000276:immunology / Q000473:pathology / Q000821:virology; D008230:Lymphomatoid Granulomatosis / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D020336:Paraparesis, Spastic / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D012074:Remission Induction; D000069283:Rituximab; D013116:Spinal Cord / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab