![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=1765911440&Signature=xVrlgK-xzPTKRY2WBov8rx0JvhYIHSwr3beM4XxZkQqnk5t37I7a-mkd52eN3IOJ-RQR5~45YpwFH3tJFOwF3FjNWeJW1UNmaxM9nzZBfsZE2ycA33CFpC4BoLgt6flvjR0T6tNLGz8x-72EJ9GVL0NU2lODz~nUMFvOL4~8BG89Uem8d79Ac1G4vsHBWyFME20XxrexZ8eohH5UQsUMV~Kem3MYQcmdsCuaE5BeAFpW0ZwlC8Gjpec4eSQ~jrEpSYPY9PF60INiDHCZ-WYt-AhoFJzwfIguFeX2q5D-KMX4zrQ3lUcBrjO1BbB1A9~T1bmU0INdR30q1t6xKfYXXQ__&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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![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=1766014136&Signature=Byvi3ItGPS5pLWzgfT5F41sbWv4JXV7iGb-oFu37vXMDVVl9VjA-9pjnKA~PQq4~VIkh7rcAlRUZ7qiIf4MyIG4lAuEN6ykQLdsudWLMidkSPkEWVhRdmlklAyZMp~DucH7Vg7QGDnTiBJbOdYQ0cej8p38KQ9Zr3EKATtS6c8i56d-rCegTe6zSzb-ocYmBbqqcYTiJ3l0EUEEWencr6iRvs8KZpD-AmRqh80I1ZXZIMj3rWNIa9M4hB1GwnBumlvfm~g8S6Qb9Q97NaYfj80Y0wDJ7ppL~b4xrtPrhMzJ36qv3U2IuGoSRpykKUKK~YVEx-rwVwm55Bij72bBSUw__&Key-Pair-Id=APKAIE5G5CRDK6RD3PGA)
