![A 33-year-old Asian woman presented with fever and neck lymphadenopathy. Laboratory examination revealed microcytic anemia, lactate dehydrogenase elevation, reverse albumin/globin ratio, and red blood cell Rouleaux formation. Serum immunofixation electrophoresis indicated polyclonal hypergammaglobinemia (immunoglobulin G [IgG] 7710 mg/dL) and a normal free light chain ratio. Other autoimmune markers were within normal limits except elevated interleukin-6. Serum IgG4/IgG ratio was low (0.12; IgG4-related disease, >0.4). Plain films of the skeleton revealed no osteolytic lesions. She had been stable and required no active treatments for 3 years. Bone marrow smear revealed atypical plasma cells (30% in total nucleated cells) (panel A) with myeloma-like features such as multiple nuclei (panel B), Russell bodies (panels C and E [arrowhead]) and flame cells (panel D) (panels A-D; original magnification ×1000; Liu stain). Bone marrow biopsy confirmed infiltrates of CD138+ plasma cells (panels E [arrow] and F). κ and λ (panel G) stains indicated no light chain restrictions. Lymph node biopsy (panel H) showed reactive interfollicular plasmacytosis. Human herpes virus-8 staining was negative (panels E and H; original magnification ×1000 [E], ×200 [H], and ×1000 [H, inset], hematoxylin and eosin stain; panels F-G; original magnification ×200, immunohistochemistry). / Idiopathic plasmacytic lymphadenopathy with polyclonal hypergammaglobinemia is a rare benign inflammatory disease and should be excluded from other autoimmune diseases, such as multicentric Castleman disease or IgG4-related disease. Clinicians should be aware of benign plasma cell proliferation mimicking malignancy.](https://ash.silverchair-cdn.com/ash/content_public/journal/blood/132/25/10.1182_blood-2018-07-863290/4/m_blood863290f1.png?Expires=1769087507&Signature=y4WJJ9kMn5Mj84JxaaokLB41aLJDHvULswVl9TJ9aQVvh5hquNjw8LBKMlf7SFb4dBM1NsHMD2Ula5g3XFmOmdP6afkVdPcMx5SWfDBmYvfl4SKHfNzsHNhjZKRqNiX0RjyKrlJFeL7HVxLmQz1T9P9G1gnR8H~idRMYPtn7~1famZJiqCo8qi7nwwxEqBlXcUb28khJL8KiLcSGtSn3xBYx2lx9VHXqHvZ-ag887mOTRguLFzO2m1ECiCwtu4ubh2IAoX69LfRs5tpp86yiFIcJ7uojBYFZR9bRT~GT6OJwwQc6iQU2BnsTGaWwxcHWtfrCT1C1EO6WZnvTPaPEoQ__&Key-Pair-Id=APKAIE5G5CRDK6RD3PGA)
A 33-year-old Asian woman presented with fever and neck lymphadenopathy. Laboratory examination revealed microcytic anemia, lactate dehydrogenase elevation, reverse albumin/globin ratio, and red blood cell Rouleaux formation. Serum immunofixation electrophoresis indicated polyclonal hypergammaglobinemia (immunoglobulin G [IgG] 7710 mg/dL) and a normal free light chain ratio. Other autoimmune markers were within normal limits except elevated interleukin-6. Serum IgG4/IgG ratio was low (0.12; IgG4-related disease, >0.4). Plain films of the skeleton revealed no osteolytic lesions. She had been stable and required no active treatments for 3 years. Bone marrow smear revealed atypical plasma cells (30% in total nucleated cells) (panel A) with myeloma-like features such as multiple nuclei (panel B), Russell bodies (panels C and E [arrowhead]) and flame cells (panel D) (panels A-D; original magnification ×1000; Liu stain). Bone marrow biopsy confirmed infiltrates of CD138+ plasma cells (panels E [arrow] and F). κ and λ (panel G) stains indicated no light chain restrictions. Lymph node biopsy (panel H) showed reactive interfollicular plasmacytosis. Human herpes virus-8 staining was negative (panels E and H; original magnification ×1000 [E], ×200 [H], and ×1000 [H, inset], hematoxylin and eosin stain; panels F-G; original magnification ×200, immunohistochemistry).
Idiopathic plasmacytic lymphadenopathy with polyclonal hypergammaglobinemia is a rare benign inflammatory disease and should be excluded from other autoimmune diseases, such as multicentric Castleman disease or IgG4-related disease. Clinicians should be aware of benign plasma cell proliferation mimicking malignancy.
A 33-year-old Asian woman presented with fever and neck lymphadenopathy. Laboratory examination revealed microcytic anemia, lactate dehydrogenase elevation, reverse albumin/globin ratio, and red blood cell Rouleaux formation. Serum immunofixation electrophoresis indicated polyclonal hypergammaglobinemia (immunoglobulin G [IgG] 7710 mg/dL) and a normal free light chain ratio. Other autoimmune markers were within normal limits except elevated interleukin-6. Serum IgG4/IgG ratio was low (0.12; IgG4-related disease, >0.4). Plain films of the skeleton revealed no osteolytic lesions. She had been stable and required no active treatments for 3 years. Bone marrow smear revealed atypical plasma cells (30% in total nucleated cells) (panel A) with myeloma-like features such as multiple nuclei (panel B), Russell bodies (panels C and E [arrowhead]) and flame cells (panel D) (panels A-D; original magnification ×1000; Liu stain). Bone marrow biopsy confirmed infiltrates of CD138+ plasma cells (panels E [arrow] and F). κ and λ (panel G) stains indicated no light chain restrictions. Lymph node biopsy (panel H) showed reactive interfollicular plasmacytosis. Human herpes virus-8 staining was negative (panels E and H; original magnification ×1000 [E], ×200 [H], and ×1000 [H, inset], hematoxylin and eosin stain; panels F-G; original magnification ×200, immunohistochemistry).
Idiopathic plasmacytic lymphadenopathy with polyclonal hypergammaglobinemia is a rare benign inflammatory disease and should be excluded from other autoimmune diseases, such as multicentric Castleman disease or IgG4-related disease. Clinicians should be aware of benign plasma cell proliferation mimicking malignancy.
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