Multicentric Castleman's disease with abundant IgG4-positive cells: a clinical and pathological analysis of six cases

Sato, Yasuharu; Kojima, Masaru; Takata, Katsuyoshi; Morito, Toshiaki; Mizobuchi, Kohichi; Tanaka, Takehiro; Inoue, Dai; Shiomi, Hideyuki; Iwao, Haruka; Yoshino, Tadashi

doi:10.1136/jcp.2010.082958

Cited by 121 publications

(88 citation statements)

References 28 publications

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“…39,[44][45][46][47][48] Many IgG4 þ plasma cells can also be present in lymphoproliferative disorders such as multicentric Castleman's disease, or even in non-specific settings such as pulmonary abscess or biliary xanthogranulomatous inflammation. 38,49 However, none of these conditions consistently shows IgG4-rich inflammation and all lack the characteristic histopathological features of IgG4-related disease. These conditions fall outside the bounds of IgG4-related disease, despite the presence in some cases of increased numbers of IgG4 þ plasma cells.…”

Section: Inflammatory Conditionsmentioning

confidence: 99%

Consensus statement on the pathology of IgG4-related disease

et al. 2012

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IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; andoften but not always-elevated serum IgG4 concentrations. An international symposium on IgG4-related The histopathology of IgG4-related disease was a specific focus of the international symposium. The primary purpose of this statement is to provide practicing pathologists with a set of guidelines for the diagnosis of IgG4-related disease. The diagnosis of IgG4-related disease rests on the combined presence of the characteristic histopathological appearance and increased numbers of IgG4 þ plasma cells. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. We propose a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy. Tissue IgG4 counts and IgG4:IgG ratios are secondary in importance. The guidelines proposed in this statement do not supplant careful clinicopathological correlation and sound clinical judgment. As the spectrum of this disease continues to expand, we advocate the use of strict criteria for accepting newly proposed entities or sites as components of the IgG4-related disease spectrum.

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Section: Inflammatory Conditionsmentioning

confidence: 99%

Consensus statement on the pathology of IgG4-related disease

et al. 2012

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“…The diagnosis of MCD is challenging because no clinical, serological, or radiological findings appear to be specific for MCD (16,17). Consequently, a biopsy of the affected lymph nodes appears to be mandatory in order to confirm the histologic characteristic findings, including numerous lymphoid follicles, active germinal centers, and interfollicular polyclonal plasmacytosis (4,16,17).…”

Section: Discussionmentioning

confidence: 99%

“…From the aspect of a serological examination, IgG4 levels are frequently elevated. Patients with multicentric Castleman disease (MCD) also occasionally have elevated serum IgG4 concentrations and abundant infiltration of IgG4-positive cells in affected organs (4)(5)(6). MCD is a polyclonal lymphoproliferative disorder accompanied by systemic symptoms that may result from increased production and circulation of interleukin-6 (IL-6) (7-9).…”

Section: Introductionmentioning

confidence: 99%

“…MCD is a polyclonal lymphoproliferative disorder accompanied by systemic symptoms that may result from increased production and circulation of interleukin-6 (IL-6) (7-9). According to previously published papers, laboratory findings including the IL-6 level, C-reactive protein (CRP) level, and platelet count appear to be important when distinguishing between IgG4-RD and MCD because an exacerbation of these inflammation parameters is less likely to be observed in patients with IgG4-RD (4,5).…”

Section: Introductionmentioning

confidence: 99%

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IgG4-related Disease Involving Multiple Organs with Elevated Serum Interleukin-6 Levels

et al. 2016

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A 63-year-old woman presented to our hospital with elevated levels of serum IgG4, marked wall thickening of the gallbladder, hepatomegaly, and abdominal lymphadenopathy. She experienced a recurrent fever and leg edema. Her laboratory data demonstrated anemia, hypoalbuminemia, and elevated serum levels of interleukin-6 and C-reactive protein. The patient was eventually diagnosed with IgG4-related disease according to the comprehensive diagnostic criteria, although the patient exhibited common clinical manifestations of multicentric Castleman disease such as a fever, anemia, lymphadenopathy, and elevated levels of serum interleukin-6 and C-reactive protein. This case report highlights the difficulties in differentiating between these two diseases.

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“…32,33 Further complicating interpretation is the observation that increased numbers of IgG4 þ plasma cells is not a finding specific to IgG4-RD. Many other benign and malignant processes may also feature increased IgG4 þ plasma cells, 27,34 including granulomatosis with polyangiitis, 35 multicentric Castleman disease, 36 Rosai-Dorfman disease, 37 marginal zone lymphoma, [38][39][40][41] pancreatic adenocarcinoma, 42,43 and even reactive lymphadenopathy, 44 to name just a few. Additionally, the histologic patterns of both these and other entities sometimes overlap with that of true IgG4-RD.…”

Section: Caveats and Differential Diagnosismentioning

confidence: 99%

IgG4-Related Disease: A Reminder for Practicing Pathologists

Weindorf

Frederiksen

2017

Archives of Pathology &Amp; Laboratory Medicine

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IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease that produces sclerotic, tumefactive masses containing dense lymphoplasmacytic infiltrates rich in immunoglobulin (Ig) G4+ plasma cells. Initially characterized as a form of autoimmune pancreatitis, the distinctive histopathology of IgG4-RD has now been described in almost every organ system. However, because the clinical manifestations of IgG4-RD are diverse and nonspecific, the disease may go unsuspected until a biopsy or resection specimen is obtained to diagnose a presumed malignancy. Pathologists thus play a key role in the diagnosis of IgG4-RD, and familiarity with its histopathologic features is essential to preventing the irreversible comorbidities associated with this treatable disease. This brief review outlines the epidemiology, clinical manifestations, and histopathology of IgG4-RD, with the aim of furthering pathologists' awareness of and ability to diagnose this disorder.

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Multicentric Castleman's disease with abundant IgG4-positive cells: a clinical and pathological analysis of six cases

Cited by 121 publications

References 28 publications

Consensus statement on the pathology of IgG4-related disease

Consensus statement on the pathology of IgG4-related disease

IgG4-related Disease Involving Multiple Organs with Elevated Serum Interleukin-6 Levels

IgG4-Related Disease: A Reminder for Practicing Pathologists

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