Clinical Implication of Idiopathic Plasmacytic Lymphadenopathy with Polyclonal Hypergammaglobulinemia: A Report of 16 Cases

Abstract

Idiopathic plasmacytic lymphadenopathy (IPL) with polyclonal hyperimmunoglobulinemia is considered identical to multicentric Castleman's disease (MCD) reported in western countries. Clinically, both IPL and MCD are characterized by multicentric lymphadenopathy, prominent polyclonal hypergammaglobulinemia, elevated erythrocyte sedimentation rate, elevated serum interleukin-6 concentration, bone marrow plasmacytosis, and various abnormal laboratory data such as anemia and positive autoantibodies. However, IPL has a significantly better 5-year survival rate than that of MCD. Moreover, none of the present 16 cases developed Kaposi's sarcoma or B-cell lymphoma. Histologically, the interfollicular area contains a sheet of polytypic mature plasma cells in both IPL and MCD. In MCD, the majority of lymphoid follicles had hyaline-vascular germinal centers. However, lymphoid follicles of IPL usually exhibit a hyperplastic germinal center. Immunostaining also demonstrated a normal/reactive follicular dendritic cell network pattern in the germinal center of IPL. Moreover, there were no human herpes virus-8-positive cells detected by immunohistochemistry. The overall clinicopathologic and immunohistochemical findings of our 16 cases suggest that IPL is distinct from MCD reported in Western countries.

Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman's disease

“…The clinical findings of systemic lymphadenopathy and hypergammaglobulinemia, which are characteristic of systemic IgG4-related lymphadenopathy, are also highly reminiscent of multicentric Castleman's disease. [15][16][17][18] However, none of our patients had anemia, hypoalbuminemia, or hypocholesterolemia, and elevated interleukin-6 and C-reactive protein were the exceptions. These findings are quite different from those of multicentric Castleman's disease.…”

“…These findings are quite different from those of multicentric Castleman's disease. [15][16][17][18] Interleukin-6 is a multifunction cytokine that has various biological activities in target cells and regulates immune responses, acute phase reactions, hematopoiesis, and bone metabolism. 25 Dysregulated overproduction of interleukin-6 is found in autoimmune diseases, such as rheumatoid arthritis, multicentric Castleman's disease, and Crohn's disease.…”

“…Idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia is considered identical to multicentric Castleman's disease in Western countries. 17,18 Idiopathic plasmacytic lymphadenopathy and multicentric Castleman's disease show similar clinicopathology. However, idiopathic plasmacytic lymphadenopathy has a significantly better 5-year survival rate than multicentric Castleman's disease.…”

“…However, idiopathic plasmacytic lymphadenopathy has a significantly better 5-year survival rate than multicentric Castleman's disease. 17,18 Multicentric Castleman's disease exhibits an aggressive and usually fatal disease course associated with infectious complications and risk of malignant tumors; one-third of such patients develop Kaposi sarcoma or B-cell lymphoma. 17,18 IgG4-related disease sometimes involves regional and/or systemic lymph nodes, 3,4,11 and is often clinically and/or histologically suspected to be multicentric Castleman's disease and/or malignant lymphoma.…”

“…17,18 Multicentric Castleman's disease exhibits an aggressive and usually fatal disease course associated with infectious complications and risk of malignant tumors; one-third of such patients develop Kaposi sarcoma or B-cell lymphoma. 17,18 IgG4-related disease sometimes involves regional and/or systemic lymph nodes, 3,4,11 and is often clinically and/or histologically suspected to be multicentric Castleman's disease and/or malignant lymphoma. 5,6,11 However, systemic IgG4-related lymphadenopathy is either not mentioned or only briefly alluded to in previous reports.…”