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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 II). (a) The lymph node shows reactive follicular hyperplasia with intact sinuses. (b) A small to moderate number of mature plasma cells with small lymphocytes and eosinophils are present. (c) Immunostaining shows numerous IgG4+ cells in the interfollicular zone.
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fig2: IgG4-related lymphadenopathy (type II). (a) The lymph node shows reactive follicular hyperplasia with intact sinuses. (b) A small to moderate number of mature plasma cells with small lymphocytes and eosinophils are present. (c) Immunostaining shows numerous IgG4+ cells in the interfollicular zone.

Mentions: The lymph nodes usually exhibit reactive follicular hyperplasia, and sinuses are intact. The reactive follicles comprise a germinal center surrounded by a discrete mantle zone. The interfollicular zone contains a small to moderate number of mature plasma cells, with small lymphocytes and eosinophils (Figure 2). This type is frequently found in the regional lymph nodes of IgG4-RD [1, 2].


IgG4-Related Lymphadenopathy.

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

IgG4-related lymphadenopathy (type II). (a) The lymph node shows reactive follicular hyperplasia with intact sinuses. (b) A small to moderate number of mature plasma cells with small lymphocytes and eosinophils are present. (c) Immunostaining shows numerous IgG4+ cells in the interfollicular zone.
© Copyright Policy - open-access
Related In: Results  -  Collection

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fig2: IgG4-related lymphadenopathy (type II). (a) The lymph node shows reactive follicular hyperplasia with intact sinuses. (b) A small to moderate number of mature plasma cells with small lymphocytes and eosinophils are present. (c) Immunostaining shows numerous IgG4+ cells in the interfollicular zone.
Mentions: The lymph nodes usually exhibit reactive follicular hyperplasia, and sinuses are intact. The reactive follicles comprise a germinal center surrounded by a discrete mantle zone. The interfollicular zone contains a small to moderate number of mature plasma cells, with small lymphocytes and eosinophils (Figure 2). This type is frequently found in the regional lymph nodes of IgG4-RD [1, 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