Objective To investigate the clinical characteristics of nodal marginal zone lymphoma (NMZL). Methods Data of 14 NMZL patients diagnosed by lymph nodes histological examination were retrospectively analyzed. Results Among the 14 patients, 13(92.9%) had abnormal complete blood counts(CBC). Leukocytosis [white blood cells(WBC)≥10.0×109/L] was observed in 6 patients (42.9%); leukocytopenia (WBC＜4.0×109/L) in 4 patients(28.6%); absolute lymphocyte counts＞5.0×109/L in 7 patients (50%); hemoglobin concentration＜120g/L in 10 patients(71.4%), thrombocytopenia(platelet＜100×109/L) in 6 patients(42.9%); cytopenia in more than 2 lineages in 4 patients (28.6%); pancytopenia in 2 cases(14.3%). Ten patients(71.4%) had bone marrow involvement. Among them, monoclonal B cells with a light chain restriction was found in 5 patients (50%). Percentage of lymphocytes in peripheral blood correlated positively with percentage of NMZL cells in bone marrow（r＝0.811，P＝0.008）. Hyperimmunoglobulinemia was found in all patients (12/12, 100%). Among them, serum monoclonal paraprotein was found in 10 patients(10/12, 83.3%). At least one auto-antibody positivity was found in 8 out of 11 patients (72.7%). All of 14 patients had Ann Arbor stage Ⅲ or Ⅳ. Immunochemotherapy was administered in 10 patients. Rituximab was given as a single agent to 1 patient. Chemotherapy was given to 2 patients, and interferon alpha was given to 1 patient. Complete response was achieved in 8 patients (57.1%), and partial response in 3 patients (21.4%). Median duration of follow-up was 23 months. Accumulated 2-year overall survival (OS) rate and 2-year progression-free survival (PFS) rate were 84.6% and 71.4%, respectively. Estimated median OS and PFS were 90 and 39 months, respectively. Conclusion NMZL patients at stage Ⅲ/Ⅳ have high a incidence of CBC abnormality, bone marrow involvement and autoimmunologic abnormality. Peripheral blood lymphocytosis might indicate NMZL bone marrow involvement.