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Immunoglobulin G4-related disease (IgG4-RD) in the orbit: mucosa-associated lymphoid tissue (MALT)-type lymphomas.

Oleś K, Składzień J, Szczepański W, Okoń K, Leszczyńska J, Bojanowska E, Bartuś K, Mika J - Med. Sci. Monit. (2015)

Bottom Line: The starting study population consisted of 167 patients with isolated infiltrative tumor diseases within the orbital region treated at the Department of Otolaryngology, Head and Neck Surgery of the Medical College Jagiellonian University in Cracow.The immunohistochemical assays using anti-IgG, anti-IgG4 and anti-CD138 antibodies were used to estimate the IgG4+/CD138+ and IgG4+/IgG+ ratios.In this study, results consistent with the criteria of IgG4-related disease were obtained in approximately 50% patients with immunohistochemical diagnosis of orbital MALT lymphoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Otolaryngology, Medical College Jagiellonian University, Cracow, Poland.

ABSTRACT

Background: MALT lymphomas were classified for differential diagnostics of IgG4-dependent disease due to their exceptional predilection to intraorbital localization. Therefore, the goal of our studies was large retrospective analysis of patients diagnosed with MALT lymphomas within the orbital tissues, since no such studies have been conducted in Poland.

Material/methods: The starting study population consisted of 167 patients with isolated infiltrative tumor diseases within the orbital region treated at the Department of Otolaryngology, Head and Neck Surgery of the Medical College Jagiellonian University in Cracow. The immunohistochemical assays using anti-IgG, anti-IgG4 and anti-CD138 antibodies were used to estimate the IgG4+/CD138+ and IgG4+/IgG+ ratios.

Results: Of all the studied and analyzed patients, a final group of 19 patients with orbital MALT lymphomas was selected to undergo diagnostic examinations for IgG4-related disease. Detailed analysis and diagnostic screening for IgG4-related disease was performed and results meeting the criteria of IgG4-dependent disease were obtained in 10 out of 19 patients with the diagnosis of MALT tumor established on the basis of immunohistochemical assays.

Conclusions: MALT lymphomas are the most common of all lymphomas occurring within orbital tissues. In this study, results consistent with the criteria of IgG4-related disease were obtained in approximately 50% patients with immunohistochemical diagnosis of orbital MALT lymphoma.

No MeSH data available.


Related in: MedlinePlus

Current international consensus diagnostic criteria for IgG4-related disease.
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f1-medscimonit-21-1043: Current international consensus diagnostic criteria for IgG4-related disease.

Mentions: Orbital tissues are the most common locations of both malignant lymphomas and inflammatory diseases. Lymphomas often bilaterally involve lacrimal glands and conjunctiva, either simultaneously or after a time interval [1,2]. Extranodal marginal B-cell lymphoma involving the mucosa-associated lymphoid tissue (MALT) is the main pathological variant of malignant lymphoma formed within orbital tissues [1,2]. Prognoses are believed to be favorable if the tumor remains in the same location for a longer time [3]. MALT lymphoma is a separate clinical nosocomial entity of characteristic histopathological features. Tumors develop as a result of prolonged antigen stimulation that might be either of autoimmune nature or due to infection, e.g. with Chlamydia psittaci; it may also be accompanied by concomitant Helicobacter pylori infection in ca. 8% of cases [4]. MALT lymphomas are diagnosed worldwide in ca 7–8% of all non-Hodgkin’s lymphoma patients worldwide. Several hypotheses have been proposed with regard to the etiology of MALT lymphomas. It is believed that there is a relationship between the tumor and the inflammation [5] because an IgG4-related disease may be a background for the development of lymphoma, particularly of MALT lymphoma within the orbital tissues [6–8]. In their recent study, Sato et al. [9] demonstrated the development of MALT lymphoma in orbital tissues on the background of chronic IgG4-dependent inflammation. Similarly, Yamamoto et al. [10] demonstrated the presence of tumors in 10.4% of patients with IgG4-related disease, i.e. ca. 3.5 times more frequently than in the overall population [10]. Marginal B-cell lymphomas producing IgG4 were described earlier [8]. This report suggests that not only the malignant tumor may occur in relation to an IgG4-related disease, but also IgG4 may be produced by cancer cells. Cheuk et al. [6] also described an IgG4-related ocular disease as a background for the development of a MALT lymphoma. The authors concluded that it was unclear whether the IgG4-related development of MALT lymphomas within the orbital tissues was due to the pre-existent IgG4-related disease or whether the observed pathology was a de novo-formed IgG4-positive MALT lymphoma. Also, other authors highlighted the difficulties in differential diagnostics between IgG4-related diseases with MALT lymphomas or other chronic inflammations. In addition, the relationship between secondary lymphomas and IgG4-related orbital diseases should be explained [11]. In the study by Go et al. [11], 14 cases of IgG4-related disease were studied, 9 of them being the MALT lymphomas, and 12 other cases consisting of other chronic inflammations of lacrimal glands and orbits. IgG4-related disease is very often associated with bilateral pathological lesions. There are no specific guidelines regarding the treatment of MALT lymphomas. The current international consensus diagnostic criteria for IgG4-related disease are as presented in Figure 1. Therapeutic options differ depending on tumor location. Therefore, diverse treatment methods are being used. Orbital IgG4-related disease is a recently reported issue that may prove important for the elucidation of the etiology of idiopathic, lymphoplasmacytic or fibrotic disorders of the orbits. Within the head, neck, and brain, the symptoms of IgG4-related orbital disease include enlargement of salivary and lacrimal glands, inflammatory orbital and salivary gland pseudotumors, pituitary changes, dura mater thickening and Riedel’s thyroiditis [12–15].


Immunoglobulin G4-related disease (IgG4-RD) in the orbit: mucosa-associated lymphoid tissue (MALT)-type lymphomas.

Oleś K, Składzień J, Szczepański W, Okoń K, Leszczyńska J, Bojanowska E, Bartuś K, Mika J - Med. Sci. Monit. (2015)

Current international consensus diagnostic criteria for IgG4-related disease.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4403377&req=5

f1-medscimonit-21-1043: Current international consensus diagnostic criteria for IgG4-related disease.
Mentions: Orbital tissues are the most common locations of both malignant lymphomas and inflammatory diseases. Lymphomas often bilaterally involve lacrimal glands and conjunctiva, either simultaneously or after a time interval [1,2]. Extranodal marginal B-cell lymphoma involving the mucosa-associated lymphoid tissue (MALT) is the main pathological variant of malignant lymphoma formed within orbital tissues [1,2]. Prognoses are believed to be favorable if the tumor remains in the same location for a longer time [3]. MALT lymphoma is a separate clinical nosocomial entity of characteristic histopathological features. Tumors develop as a result of prolonged antigen stimulation that might be either of autoimmune nature or due to infection, e.g. with Chlamydia psittaci; it may also be accompanied by concomitant Helicobacter pylori infection in ca. 8% of cases [4]. MALT lymphomas are diagnosed worldwide in ca 7–8% of all non-Hodgkin’s lymphoma patients worldwide. Several hypotheses have been proposed with regard to the etiology of MALT lymphomas. It is believed that there is a relationship between the tumor and the inflammation [5] because an IgG4-related disease may be a background for the development of lymphoma, particularly of MALT lymphoma within the orbital tissues [6–8]. In their recent study, Sato et al. [9] demonstrated the development of MALT lymphoma in orbital tissues on the background of chronic IgG4-dependent inflammation. Similarly, Yamamoto et al. [10] demonstrated the presence of tumors in 10.4% of patients with IgG4-related disease, i.e. ca. 3.5 times more frequently than in the overall population [10]. Marginal B-cell lymphomas producing IgG4 were described earlier [8]. This report suggests that not only the malignant tumor may occur in relation to an IgG4-related disease, but also IgG4 may be produced by cancer cells. Cheuk et al. [6] also described an IgG4-related ocular disease as a background for the development of a MALT lymphoma. The authors concluded that it was unclear whether the IgG4-related development of MALT lymphomas within the orbital tissues was due to the pre-existent IgG4-related disease or whether the observed pathology was a de novo-formed IgG4-positive MALT lymphoma. Also, other authors highlighted the difficulties in differential diagnostics between IgG4-related diseases with MALT lymphomas or other chronic inflammations. In addition, the relationship between secondary lymphomas and IgG4-related orbital diseases should be explained [11]. In the study by Go et al. [11], 14 cases of IgG4-related disease were studied, 9 of them being the MALT lymphomas, and 12 other cases consisting of other chronic inflammations of lacrimal glands and orbits. IgG4-related disease is very often associated with bilateral pathological lesions. There are no specific guidelines regarding the treatment of MALT lymphomas. The current international consensus diagnostic criteria for IgG4-related disease are as presented in Figure 1. Therapeutic options differ depending on tumor location. Therefore, diverse treatment methods are being used. Orbital IgG4-related disease is a recently reported issue that may prove important for the elucidation of the etiology of idiopathic, lymphoplasmacytic or fibrotic disorders of the orbits. Within the head, neck, and brain, the symptoms of IgG4-related orbital disease include enlargement of salivary and lacrimal glands, inflammatory orbital and salivary gland pseudotumors, pituitary changes, dura mater thickening and Riedel’s thyroiditis [12–15].

Bottom Line: The starting study population consisted of 167 patients with isolated infiltrative tumor diseases within the orbital region treated at the Department of Otolaryngology, Head and Neck Surgery of the Medical College Jagiellonian University in Cracow.The immunohistochemical assays using anti-IgG, anti-IgG4 and anti-CD138 antibodies were used to estimate the IgG4+/CD138+ and IgG4+/IgG+ ratios.In this study, results consistent with the criteria of IgG4-related disease were obtained in approximately 50% patients with immunohistochemical diagnosis of orbital MALT lymphoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Otolaryngology, Medical College Jagiellonian University, Cracow, Poland.

ABSTRACT

Background: MALT lymphomas were classified for differential diagnostics of IgG4-dependent disease due to their exceptional predilection to intraorbital localization. Therefore, the goal of our studies was large retrospective analysis of patients diagnosed with MALT lymphomas within the orbital tissues, since no such studies have been conducted in Poland.

Material/methods: The starting study population consisted of 167 patients with isolated infiltrative tumor diseases within the orbital region treated at the Department of Otolaryngology, Head and Neck Surgery of the Medical College Jagiellonian University in Cracow. The immunohistochemical assays using anti-IgG, anti-IgG4 and anti-CD138 antibodies were used to estimate the IgG4+/CD138+ and IgG4+/IgG+ ratios.

Results: Of all the studied and analyzed patients, a final group of 19 patients with orbital MALT lymphomas was selected to undergo diagnostic examinations for IgG4-related disease. Detailed analysis and diagnostic screening for IgG4-related disease was performed and results meeting the criteria of IgG4-dependent disease were obtained in 10 out of 19 patients with the diagnosis of MALT tumor established on the basis of immunohistochemical assays.

Conclusions: MALT lymphomas are the most common of all lymphomas occurring within orbital tissues. In this study, results consistent with the criteria of IgG4-related disease were obtained in approximately 50% patients with immunohistochemical diagnosis of orbital MALT lymphoma.

No MeSH data available.


Related in: MedlinePlus