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IgG4-Related Lymphadenopathy.

Sato Y, Yoshino T - Int J Rheumatol (2012)

Bottom Line: Laboratory analyses are crucial to differentiate between the 2 diseases.In contrast, IgG4-RD does not share any of these characteristics.Therefore, the diagnosis of IgG4-RD requires not only pathological findings but also clinical and laboratory analyses.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.

ABSTRACT
Lymphadenopathy is frequently observed in patients with immunoglobulin G4-related disease (IgG4-RD) and sometimes appears as the first manifestation of the disease. The diagnosis of IgG4-related lymphadenopathy is complicated owing to a great histological diversity, with at least 5 histological subtypes. Indeed, lymph node biopsy may be performed under the suspicion that the lymphadenopathy is a malignant lymphoma or other lymphoproliferative disorder. The diagnosis of IgG4-RD is characterized by both elevated serum IgG4 (>135 mg/dL) and histopathological features, including a dense lymphoplasmacytic infiltrate rich in IgG4(+) plasma cells (IgG4(+)/IgG(+) plasma cell ratio >40%). However, patients with hyper-interleukin (IL-) 6 syndromes such as multicentric Castleman's disease, rheumatoid arthritis, and other immune-mediated conditions frequently show lymph node involvement and often fulfill the diagnostic criteria for IgG4-RD. Owing to these factors, IgG4-RD cannot be differentiated from hyper-IL-6 syndromes on the basis of histological findings alone. Laboratory analyses are crucial to differentiate between the 2 diseases. Hyper-IL-6 syndromes are characterized by elevated serum levels of IgG, IgA, IgM, and C-reactive protein (CRP); thrombocytosis; anemia; hypoalbuminemia; hypocholesterolemia. In contrast, IgG4-RD does not share any of these characteristics. Therefore, the diagnosis of IgG4-RD requires not only pathological findings but also clinical and laboratory analyses.

No MeSH data available.


Related in: MedlinePlus

IgG4-related lymphadenopathy (type I). (a) The lymph node shows interfollicular expansion with normal to hyperplastic germinal centers. (b) The germinal centers are penetrated by blood vessels. (c) A large number of mature plasma cells with small lymphocytes are seen. (d) Immunostaining shows numerous IgG4+ cells in the interfollicular zone.
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fig1: IgG4-related lymphadenopathy (type I). (a) The lymph node shows interfollicular expansion with normal to hyperplastic germinal centers. (b) The germinal centers are penetrated by blood vessels. (c) A large number of mature plasma cells with small lymphocytes are seen. (d) Immunostaining shows numerous IgG4+ cells in the interfollicular zone.

Mentions: This type is frequently characterized by systemic lymphadenopathy [1–3]. Histologically, the lymph node shows interfollicular expansion with normal to hyperplastic germinal centers, penetrated by blood vessels. Abundant plasma cells and scattered eosinophils are apparent in the interfollicular zone (Figure 1). Although these features are similar to the features of multicentric Castleman's disease (MCD), MCD is usually characterized by the presence of small and regressive germinal centers and no eosinophil infiltration [8]. However, pathological diagnosis is difficult, because MCD sometimes fulfills the diagnostic criteria for IgG4-RD, namely, abundant IgG4+ plasma cell infiltration (i.e., IgG4+/IgG+ plasma cell ratio >40%) and elevated serum IgG4 levels [8]. Therefore, the 2 diseases cannot be differentiated on the basis of histological findings alone, and laboratory analyses are critical for a definitive diagnosis (Table 2).


IgG4-Related Lymphadenopathy.

Sato Y, Yoshino T - Int J Rheumatol (2012)

IgG4-related lymphadenopathy (type I). (a) The lymph node shows interfollicular expansion with normal to hyperplastic germinal centers. (b) The germinal centers are penetrated by blood vessels. (c) A large number of mature plasma cells with small lymphocytes are seen. (d) Immunostaining shows numerous IgG4+ cells in the interfollicular zone.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3376648&req=5

fig1: IgG4-related lymphadenopathy (type I). (a) The lymph node shows interfollicular expansion with normal to hyperplastic germinal centers. (b) The germinal centers are penetrated by blood vessels. (c) A large number of mature plasma cells with small lymphocytes are seen. (d) Immunostaining shows numerous IgG4+ cells in the interfollicular zone.
Mentions: This type is frequently characterized by systemic lymphadenopathy [1–3]. Histologically, the lymph node shows interfollicular expansion with normal to hyperplastic germinal centers, penetrated by blood vessels. Abundant plasma cells and scattered eosinophils are apparent in the interfollicular zone (Figure 1). Although these features are similar to the features of multicentric Castleman's disease (MCD), MCD is usually characterized by the presence of small and regressive germinal centers and no eosinophil infiltration [8]. However, pathological diagnosis is difficult, because MCD sometimes fulfills the diagnostic criteria for IgG4-RD, namely, abundant IgG4+ plasma cell infiltration (i.e., IgG4+/IgG+ plasma cell ratio >40%) and elevated serum IgG4 levels [8]. Therefore, the 2 diseases cannot be differentiated on the basis of histological findings alone, and laboratory analyses are critical for a definitive diagnosis (Table 2).

Bottom Line: Laboratory analyses are crucial to differentiate between the 2 diseases.In contrast, IgG4-RD does not share any of these characteristics.Therefore, the diagnosis of IgG4-RD requires not only pathological findings but also clinical and laboratory analyses.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.

ABSTRACT
Lymphadenopathy is frequently observed in patients with immunoglobulin G4-related disease (IgG4-RD) and sometimes appears as the first manifestation of the disease. The diagnosis of IgG4-related lymphadenopathy is complicated owing to a great histological diversity, with at least 5 histological subtypes. Indeed, lymph node biopsy may be performed under the suspicion that the lymphadenopathy is a malignant lymphoma or other lymphoproliferative disorder. The diagnosis of IgG4-RD is characterized by both elevated serum IgG4 (>135 mg/dL) and histopathological features, including a dense lymphoplasmacytic infiltrate rich in IgG4(+) plasma cells (IgG4(+)/IgG(+) plasma cell ratio >40%). However, patients with hyper-interleukin (IL-) 6 syndromes such as multicentric Castleman's disease, rheumatoid arthritis, and other immune-mediated conditions frequently show lymph node involvement and often fulfill the diagnostic criteria for IgG4-RD. Owing to these factors, IgG4-RD cannot be differentiated from hyper-IL-6 syndromes on the basis of histological findings alone. Laboratory analyses are crucial to differentiate between the 2 diseases. Hyper-IL-6 syndromes are characterized by elevated serum levels of IgG, IgA, IgM, and C-reactive protein (CRP); thrombocytosis; anemia; hypoalbuminemia; hypocholesterolemia. In contrast, IgG4-RD does not share any of these characteristics. Therefore, the diagnosis of IgG4-RD requires not only pathological findings but also clinical and laboratory analyses.

No MeSH data available.


Related in: MedlinePlus