Abstract
This paper reports a case of idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia (IPL) resembling systemic lupus erythematosus (SLE). A 48-year-old lady was hospitalized with complaints of abdominal pain and high fever. She was found to have a pleural effusion in the right side, hepatosplenomegaly, general lymphadenopathy, and Raynaud's phenomenon. Laboratory examination revealed the followings: ESR 125mm/hr, Hb 9.5g/dl, WBC 5, 400/μl, platelet 1.6×104/μl, creatinine 3.0mg/dl, amylase 622 IU/l, γ-globulin 4.19g/dl, CRP 15.2mg/dl, CH 50 less than 12.0U/ml, rheumatoid factor 148.9IU/ml, negative tests for LE cells or antinuclear antibody. A biopsy of the cervical lymphnodes revealed a marked increase of plasma cells with preserved follicular structures. She was diagnosed as IPL, and successfully treated with the combination chemotherapy (vincristine 1.5mg, cyclophosphamide 500mg and prednisolone). IPL is a distinct clinical entity, characterized by systemic lymphadenopathy and marked polyclonal hyperimmunoglobulinemia. The disorder must be distinguished from other diseases, in the spectrum of lymphadenopathies from angioimmunoblastic lymphadenopathy-related disorder to autoimmune-associated lymphadenopathy, most notably SLE.
