0
Signs and Symptoms of Chest Diseases |

Concomitant IgG4-Lymphadenopathy in a Patient With Diffuse Large B-Cell Lymphoma

Aarti Mittal, DO; Sara Acree, MD; Ching-Fei Chang, MD
Author and Funding Information

University of Southern California, Division of Pulmonary, Critical Care, and Sleep Medicine, Los Angeles, CA


Chest. 2013;144(4_MeetingAbstracts):928A. doi:10.1378/chest.1703197
Text Size: A A A
Published online

Abstract

SESSION TITLE: Miscellaneous Cases III

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Sunday, October 27, 2013 at 03:00 PM - 04:00 PM

INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is a newly discovered inflammatory disorder characterized by infiltration of key organs by IgG4-positive plasma cells. It typically presents with systemic lymphadenopathy in the setting of autoimmune pancreatitis and sclerosing sialadenitis, but isolated lymphadenopathy is now being increasingly recognized as an early manifestation of this disease. We present a patient with mediastinal and right hilar lymphadenopathy thought to be due to recurrence of his diffuse large B-cell lymphoma (DLBCL) until mediastinoscopy confirmed reactive lymphoid hyperplasia with unexpected findings of IgG4 plasmacytosis.

CASE PRESENTATION: A 62-year-old man with a history of Stage IV DLBCL manifesting as a chest wall mass presented with a surveillance computerized tomography (CT) scan showing enlarging mediastinal and right hilar lymph nodes. FDG-PET activity was surprisingly low and serum LDH was 124. Mediastinoscopy revealed marked IgG plasmocytosis comprised almost exclusively of IgG4. Serum IgG4:IgG ratio and IgE levels were both significantly elevated, but IL-6 levels were normal. Collagen-vascular disease serologies were unremarkable. The original chest wall mass biopsy was re-reviewed, but did not show increased levels of IgG4. Therefore, this patient was diagnosed with probable IgG4 lymphadenopathy and given high-dose steroids with good response.

DISCUSSION: IgG4-lymphadenopathy is considered a sub-category IgG4-RD in which no other organ is involved. It is characterized by marked IgG4 plasmocytosis in lymph nodes, a serum IgG4:IgG of greater than 40%, and elevated IgE levels. Mass effect on surrounding organs is the main issue; otherwise, this disease is relatively benign. Because the clinical presentation of IgG4-lymphadenopathy can mimic lymphoma, Castleman’s disease, and hyper-IL-6 autoimmune conditions, IL-6, and CRP levels should be ordered to help exclude these. In contrast to IgG4-RD in other organs, findings of storiform fibrosis and obliterative phlebitis are usually absent. Instead, five different histological subtypes are increasingly being recognized, which complicates the diagnosis: Multicentric Castleman’s Disease-Like, Follicular Hyperplasia, Interfollicular Expansion, Progressively Transformed Germinal Centers-Like, and Inflammatory Pseudotumor-Like. However, regardless of the histologic subtype, all forms of IgG4-lymphadenopathy respond readily to glucocorticoids with an excellent prognosis overall.

CONCLUSIONS: Our patient was initially suspected to have mediastinal recurrence of his DLBCL, but was found to have a completely different disease (IgG4-lymphadenopathy) on subsequent biopsy. This underscores the importance of pursuing tissue confirmation in the setting of prior lymphoma to exclude other potential etiologies which may obviate the need for toxic chemoradiotherapy and significantly affect prognosis.

Reference #1: Sato et al. International Journal of Rheumatology 2012

DISCLOSURE: The following authors have nothing to disclose: Aarti Mittal, Sara Acree, Ching-Fei Chang

No Product/Research Disclosure Information


Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Figures

Tables

References

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543