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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 IV). (a) The lymph node shows marked follicular hyperplasia with PTGC. (b) The PTGCs appear as round to oval structures 2-3 times the diameter of the other reactive follicles. (c) Numerous eosinophils infiltrate the interfollicular zone. (d) The majority of IgG4+ plasma cells reside in the germinal centers, with a small number present in the interfollicular zone. (e), (f): The IgG4+/IgG+ plasma cell ratio is >40% (e: IgG4-immunostain, f: IgG-immunostain).
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fig4: IgG4-related lymphadenopathy (type IV). (a) The lymph node shows marked follicular hyperplasia with PTGC. (b) The PTGCs appear as round to oval structures 2-3 times the diameter of the other reactive follicles. (c) Numerous eosinophils infiltrate the interfollicular zone. (d) The majority of IgG4+ plasma cells reside in the germinal centers, with a small number present in the interfollicular zone. (e), (f): The IgG4+/IgG+ plasma cell ratio is >40% (e: IgG4-immunostain, f: IgG-immunostain).

Mentions: PTGC is a benign condition of unknown origin characterized by reactive follicular hyperplasia in the lymph nodes [10, 11]. Recently, we were the first to report cases of patients with IgG4-RD in PTGC of lymph nodes (PTGC-type IgG4-related lymphadenopathy) [3]. In this type, the lymph nodes demonstrate numerous lymphoid follicles with hyperplastic germinal centers and a distinct mantle zone but no expansion of the interfollicular zone. PTGCs are also apparent, appearing as round to oval structures with diameters 2 or 3 times the diameter of the other reactive follicles. They are predominantly composed of small lymphocytes, centrocytes, centroblasts, and numerous mature plasma cells and plasmacytoid cells. The interfollicular zone shows infiltration by numerous eosinophils, whereas T zones are indistinct (Figure 4). Interestingly, a unique feature of this type is the localization of the majority of IgG4+ plasma cells in the germinal centers, with only a small number present in the interfollicular zone [12]. However, in a few cases of this type, IgG4+ plasma cells are detected in both the germinal centers and interfollicular zone [12].


IgG4-Related Lymphadenopathy.

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

IgG4-related lymphadenopathy (type IV). (a) The lymph node shows marked follicular hyperplasia with PTGC. (b) The PTGCs appear as round to oval structures 2-3 times the diameter of the other reactive follicles. (c) Numerous eosinophils infiltrate the interfollicular zone. (d) The majority of IgG4+ plasma cells reside in the germinal centers, with a small number present in the interfollicular zone. (e), (f): The IgG4+/IgG+ plasma cell ratio is >40% (e: IgG4-immunostain, f: IgG-immunostain).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig4: IgG4-related lymphadenopathy (type IV). (a) The lymph node shows marked follicular hyperplasia with PTGC. (b) The PTGCs appear as round to oval structures 2-3 times the diameter of the other reactive follicles. (c) Numerous eosinophils infiltrate the interfollicular zone. (d) The majority of IgG4+ plasma cells reside in the germinal centers, with a small number present in the interfollicular zone. (e), (f): The IgG4+/IgG+ plasma cell ratio is >40% (e: IgG4-immunostain, f: IgG-immunostain).
Mentions: PTGC is a benign condition of unknown origin characterized by reactive follicular hyperplasia in the lymph nodes [10, 11]. Recently, we were the first to report cases of patients with IgG4-RD in PTGC of lymph nodes (PTGC-type IgG4-related lymphadenopathy) [3]. In this type, the lymph nodes demonstrate numerous lymphoid follicles with hyperplastic germinal centers and a distinct mantle zone but no expansion of the interfollicular zone. PTGCs are also apparent, appearing as round to oval structures with diameters 2 or 3 times the diameter of the other reactive follicles. They are predominantly composed of small lymphocytes, centrocytes, centroblasts, and numerous mature plasma cells and plasmacytoid cells. The interfollicular zone shows infiltration by numerous eosinophils, whereas T zones are indistinct (Figure 4). Interestingly, a unique feature of this type is the localization of the majority of IgG4+ plasma cells in the germinal centers, with only a small number present in the interfollicular zone [12]. However, in a few cases of this type, IgG4+ plasma cells are detected in both the germinal centers and interfollicular zone [12].

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