Increased IgG4‐positive plasma cells in nodular‐sclerosing Hodgkin lymphoma: a diagnostic pitfall

Nowak, Verena; Agaimy, Abbas; Kristiansen, Glen; Gütgemann, Ines

doi:10.1111/his.13965

Cited by 13 publications

(3 citation statements)

References 20 publications

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“…The pathological mimickers of AIP or IgG4-related diseases, such as Castleman's disease [47], follicular pancreatitis [48], anti-neutrophil cytoplasmic antibody-associated vasculitides [49,50], inflammatory myofibroblastic tumor [51], and lymphoma [52], are listed from the viewpoints of lymphoplasmacytic proliferation.…”

Section: Mimickers Of Aipmentioning

confidence: 99%

Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

Matsubayashi

Ishiwatari

Imai

et al. 2019

IJMS

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Autoimmune pancreatitis (AIP), a unique subtype of pancreatitis, is often accompanied by systemic inflammatory disorders. AIP is classified into two distinct subtypes on the basis of the histological subtype: immunoglobulin G4 (IgG4)-related lymphoplasmacytic sclerosing pancreatitis (type 1) and idiopathic duct-centric pancreatitis (type 2). Type 1 AIP is often accompanied by systemic lesions, biliary strictures, hepatic inflammatory pseudotumors, interstitial pneumonia and nephritis, dacryoadenitis, and sialadenitis. Type 2 AIP is associated with inflammatory bowel diseases in approximately 30% of cases. Standard therapy for AIP is oral corticosteroid administration. Steroid treatment is generally indicated for symptomatic cases and is exceptionally applied for cases with diagnostic difficulty (diagnostic steroid trial) after a negative workup for malignancy. More than 90% of patients respond to steroid treatment within 1 month, and most within 2 weeks. The steroid response can be confirmed on clinical images (computed tomography, ultrasonography, endoscopic ultrasonography, magnetic resonance imaging, and 18F-fluorodeoxyglucose-positron emission tomography). Hence, the steroid response is included as an optional diagnostic item of AIP. Steroid treatment results in normalization of serological markers, including IgG4. Short- and long-term corticosteroid treatment may induce adverse events, including chronic glycometabolism, obesity, an immunocompromised status against infection, cataracts, glaucoma, osteoporosis, and myopathy. AIP is common in old age and is often associated with diabetes mellitus (33–78%). Thus, there is an argument for corticosteroid therapy in diabetes patients with no symptoms. With low-dose steroid treatment or treatment withdrawal, there is a high incidence of AIP recurrence (24–52%). Therefore, there is a need for long-term steroid maintenance therapy and/or steroid-sparing agents (immunomodulators and rituximab). Corticosteroids play a critical role in the diagnosis and treatment of AIP.

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Section: Mimickers Of Aipmentioning

confidence: 99%

Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

Matsubayashi

Ishiwatari

Imai

et al. 2019

IJMS

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“…IgG4-RD, as a rare multiorgan immune-mediated fibrotic inflammatory disease undefined until 2001 ( 14 ), is typically characterized by the presence of a dense IgG4-positive plasma cell infiltrate, the presence of mild to moderate eosinophilic infiltrate, extracellular matrix fibrosis and occlusive phlebitis ( 15 ). Organs considered typical for IgG4-RD infiltration are the lacrimal glands, major salivary glands, orbits, lungs, paravertebral soft tissue, pancreas, biliary tree, kidneys, retroperitoneum, aorta, meninges and thyroid gland ( 6 ).…”

Section: Discussionmentioning

confidence: 99%

Myelodysplastic syndrome with IgG4‑related disease: A case report

Wang,

Pang,

Hong

et al. 2024

Oncol Lett

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At present, to the best of our knowledge, there are only a few case reports of IgG4-related disease (IgG4-RD) involving myelodysplastic syndrome (MDS), yet the incidence of MDS and IgG4-RD is increasing in middle-aged and elderly people. The present study presents a case of MDS combined with IgG4-RD admitted to Zhejiang Provincial Hospital of Chinese Medicine in September 2022. The (66-year-old; male) patient was admitted to the hospital due to hematopenia with an elevated IgG4 index. The diagnosis of MDS combined with IgG4-RD was confirmed after various exams, including pathological examination. The condition of the patient improved after 3 weeks of hormone therapy, with a significant increase in complete blood count compared with the pre-treatment period. MDS is a malignant hematological disorder with a high risk of conversion to leukemia, and IgG4-RD is a systemic immune-mediated disease with a poor prognosis often associated with malignancy. The present study presents and reviews the literature to better understand the coexistence of these two diseases.

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“…However, lymph nodes, the lung, and other organs and tissues often do not have the characteristic manifestations of storiform-type fibrosis and phlebitis obliterans. IgG4-correlated disease in the lymph nodes is easily misdiagnosed due to the lack of specificity of its clinical pathological features, which have been identified in a variety of pathological conditions, such as Castleman disease, inflammatory pseudotumor, and lymphoid follicle hyperplasia of reactivity (such as lymphoma), and final diagnosis should combine medical history, physical examination, serological examination, imaging, pathology, and immunohistochemistry[ 9 - 11 ].…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease: A case report

Hao¹,

Yang²,

Bian³

et al. 2021

WJCC

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BACKGROUND Immunoglobulin (Ig) G4-associated diseases are a group of systemic diseases involving multiple organs and are also known as IgG4-associated sclerosing diseases. IgG4-associated lymphadenopathy occurring in the lymph nodes is characterized by a lack of specificity due to its clinicopathological characteristics and must be differentiated from a variety of lesions, such as Castleman disease, lymphatic follicular reactive hyperplasia, and lymphoma. CASE SUMMARY A 65-year-old male patient, with Guillain-Barre syndrome for 5 years, presented to our hospital complaining of bilateral orbital mass for 2 years. After hospitalization, the results of the patient’s laboratory tests showed that immunoglobulin subgroup IgG4 was 33.90 g/L and IgG was 30.30 g/L, but serum interleukin-6 was normal. The pathological morphology of orbital mass and cervical lymph node were consistent, which showed that a large number of plasma cells and eosinophils were observed in the lymphatic follicles, and the interstitial fibrous tissue was proliferative. Immunohistochemistry showed that CD20 (B cells) (+), CD3 (T cells) (+), CD38 (+), IgG (+), IgG4 positive cells > 100/high powered field, and IgG4/IgG > 40%. Combined with clinical and immunohistochemical results, lymphadenopathy was consistent with Castleman disease-like IgG4-associated sclerosing disease. Prednisone acetate treatment was given at 40 mg/d. After 2 wk, the superficial lymph nodes and orbital masses shrank, and the IgG4 level decreased. As prednisone acetate was regularly used at a reduced dosage, no recurrence of the disease has been observed. CONCLUSION This case suggested that it is necessary to proceed cautiously in clinical practice with such patients, and immunoglobulin, complement, interleukin-6, C-reactive protein, and other examinations should be performed to confirm the diagnosis.

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Increased IgG4‐positive plasma cells in nodular‐sclerosing Hodgkin lymphoma: a diagnostic pitfall

Cited by 13 publications

References 20 publications

Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

Myelodysplastic syndrome with IgG4‑related disease: A case report

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease: A case report

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