Abstract
BackgroundCentral nervous system (CNS) lesions and peripheral neuropathy are rare among non-Hodgkin’s lymphoma (NHL) patients. Usually, lymphoma infiltration or local oppression account for CNS or peripheral nerve lesions. The incidence of peripheral neuropathy was reported to be 5%. Guillain-Barre Syndrome (GBS) is rarer and may occur in less than 0.3% of NHL patients. Hemophagocytic syndrome (HPS) is another rare complication of NHL. It was reported that 1% of patients with hematological malignancies suffer from HPS. Diffuse large B cell lymphoma (DLBCL) combined with GBS has been reported in 10 cases to date. Case presentationHerein, we reported the case of a 53-year-old man, who was initially hospitalized for abnormal feeling in lower limbs and urinary incontinence. He was finally diagnosed as DLBCL combined with GBS and HPS after 16 days, which is earlier than previously reported. Immunoglobulin pulse therapy, dexamethasone and etoposide were immediately administered. The neurological symptoms did not improve obviously, but cytopenia was relieved. However, GBS-related clinical manifestations partially recovered after one cycle of R-CHOP (Rituximab-Cyclophosphamide, Hydroxydaunorubicin, vincristine, Prednisone) chemotherapy, and disappeared after six cycles of R-CHOP. ConclusionsLiterature review indicated that DLBCL patients combined with GBS were usually elderly, more than 80% of patients were male and over 60 years old. GBS could occur before lymphoma is diagnosed or occur after lymphoma is diagnosed or treated. Pathogenesis of GBS remains unclear. Molecular simulation is considered as the main mechanism. For such patients, tumor-related immune activation may be the main pathogenesis, and chemotherapy for lymphoma may be crucial. GBS and HPS heralding the diagnosis of EBV DLBCL is clinically rare. Herein, we reported a rare case and shared our clinical experience. If GBS occurs before lymphoma diagnosis, traditional therapies may be effective. Rapid diagnosis and timely treatment of DLBCL are crucial.
Diffuse large B-Cell lymphoma associated with paraneoplastic Guillain–Barré syndrome: A diagnostic and therapeutic challenge
