Lymphadenopathy of IgG4-related sclerosing disease: three case reports and review of literature.
Immunoglobulin (Ig) G4-related sclerosing disease is a recently described syndrome characterized by lymphoplasmacytic infiltration of exocrine glands or extranodal tissues and elevated serum IgG4. We report three cases of lymphadenopathy secondary to IgG4-related sclerosing disease. Histologic features of involved lymph nodes included interfollicular immunoblasts and plasma cells, similar to Castleman's disease. The percentage of IgG4-/IgG-positive plasma cells in the three patients was markedly elevated (30, 50, and 60%). Administration of prednisolone led to remission in every case. One of three cases was consulted to our hospital due to suspected diagnosis of angioimmunoblastic T cell lymphoma (AITL). The case demonstrates many clinical and pathologic similarities between IgG4-related sclerosing disease and AITL. Pathological similarities between AITL and the lymphoplasmacytic subtype of IgG4-sclerosing disease have recently been reported. It is important to accurately diagnose IgG4-related lymphadenopathy given its ready response to steroid therapy and the potential for misdiagnosing lymphoma on clinical grounds.
Department of Hematology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano, Nagano 380-8582, Japan. iqo430@kc4.so-net.ne.jp
Ikuo
I
Shimizu
Kentaro
K
Nasu
Keijiro
K
Sato
Hiromitsu
H
Ueki
Daigo
D
Akahane
Masahiko
M
Sumi
Mayumi
M
Ueno
Naoaki
N
Ichikawa
Naoko
N
Asano
Masaru
M
Kojima
Hikaru
H
Kobayashi
D000368:Aged; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology; D008206:Lymphatic Diseases / Q000473:pathology*; D008221:Lymphoid Tissue / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000473:pathology; D008297:Male; D010950:Plasma Cells / Q000166:cytology; D011241:Prednisone / Q000627:therapeutic use; D012598:Sclerosis / Q000188:drug therapy / Q000473:pathology*; D016896:Treatment Outcome
D005938:Glucocorticoids; D007074:Immunoglobulin G; D011241:Prednisone