Successful treatment of systemic and central nervous system lymphomatoid granulomatosis with rituximab.
Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder with a mortality rate approaching 60% in the first year. The median survival is 14 months from the time of diagnosis. Although a variety of chemotherapeutic regimens have been utilized, there is no standard treatment. Studies have shown that in most cases the malignant cells are B-cells, which induce massive infiltration of reactive T-lymphocytes in the background. The disease is therefore considered as a T-cell rich B-cell lymphoproliferative disorder. We report a case of LYG with pulmonary, hepatic, central and peripheral nervous system involvement that was successfully treated with the anti-CD20 (B-cell) monoclonal antibody, Rituximab.
Zaidi
A
A
Department of Medicine, Division of Neoplastic Diseases and Related Disorders, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
Kampalath
B
B
Peltier
W L
WL
Vesole
D H
DH
D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001402:B-Lymphocytes; D016543:Central Nervous System Neoplasms / Q000188:drug therapy*; D018572:Disease-Free Survival; D006801:Humans; D007150:Immunohistochemistry; D008230:Lymphomatoid Granulomatosis / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000473:pathology; D012074:Remission Induction; D000069283:Rituximab; D013601:T-Lymphocytes; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab