IgG4-related lymphadenopathy – a difficult diagnosis

2020 ◽  
Vol 3 (1) ◽  
Author(s):  
Balan Louis Gaspar
Keyword(s):  
Lymph Node ◽  
Soft Tissue ◽  
Plasma Cells ◽  
Lymph Node Excision ◽  
Excision Biopsy ◽  
Neoplastic Disease ◽  
Anatomical Site ◽  
Axillary Lymph ◽  
Luminal Narrowing ◽  
Serum Igg4

Abstract Background The IgG4-related disease (IgG4-RD) is a systemic immune-mediated non-neoplastic disease associated with IgG4 positive plasma cells and fibrosis that often presents as a mass lesion. Although the disease could affect virtually any anatomical site, there are strong predilections for certain organs. IgG4-related lymphadenopathy can exhibit a broad morphologic spectrum. We describe a patient with IgG4-related lymphadenopathy with overlapping histological features that proved to be a diagnostic red herring. Case presentation A 58-year-old gentleman with multiple co-morbidities presented with obstructive jaundice, elevated transaminases, and bilateral inguinal and left axillary lymphadenopathy. Imaging of the abdomen and pelvis showed circumferential soft tissue thickening resulting in luminal narrowing of common and proximal bile duct with upstream intrahepatic biliary radicle dilatation, multiple enlarged lymph nodes, and homogenous soft tissue lesions in the tail of the pancreas and bilateral renal cortical parenchyma with perinephric soft tissue extension. Left inguinal, and axillary lymph node excision biopsies were suggestive of IgG4-RD. Serum IgG4 levels performed subsequently, were markedly elevated. The patient was treated with prednisone which led to resolutions of his symptoms, reduction in the size of the lesions, and reversal of abnormal laboratory parameters. Conclusion The diagnosis of IgG4-RD in lymph node excision biopsy is a difficult call to make and needs a multidisciplinary team. An early diagnosis renders timely intervention and prevents the progression of the disease and its complications.

scholarly journals IgG4-Related Lymphadenopathy

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Yasuharu Sato ◽  
Tadashi Yoshino
Keyword(s):  
Lymph Node ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Serum Levels ◽  
Lymph Node Involvement ◽  
Lymph Node Biopsy ◽  
Immunoglobulin G4 ◽  
Reactive Protein ◽  
Serum Igg4 ◽  
Node Involvement

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.

scholarly journals A Retrospectively Diagnosed Case of IgG4-Related Tubulointerstitial Nephritis Showing Good Renal Outcome and Pathological Progress

2013 ◽  
Vol 2013 ◽  
pp. 1-7
Author(s):  
Qiong Wu ◽  
Raima Nakazawa ◽  
Hisae Tanaka ◽  
Masayuki Endoh ◽  
Masafumi Fukagawa
Keyword(s):  
Diabetic Nephropathy ◽  
Lymph Node ◽  
Plasma Cells ◽  
Tubulointerstitial Nephritis ◽  
Lymph Node Involvement ◽  
Renal Outcome ◽  
Creatinine Concentration ◽  
Chronic Sialadenitis ◽  
Serum Igg4 ◽  
Node Involvement

A 74-year-old man was hospitalized for diabetic nephropathy evaluation and assessment of the effect of treatment on his tubulointerstitial nephritis (TIN). When he was 62 years old, he developed polyarthralgia and had superficial lymph node swelling, mildly increased serum creatinine concentration, hypergammaglobulinemia, hypocomplementemia, high serum IL-2R level, and positive titer of antinuclear antibody. Several tissues were biopsied. Mild chronic sialadenitis and reactive lymphadenitis were identified. Renal specimen showed mild glomerular ischemia, extensive storiform fibrosis, and abundant infiltrating monocytes and plasma cells. He was treated with oral prednisolone and cyclophosphamide. After the treatment, most of his clinical parameters quickly returned to within the reference range. However, he developed diabetes mellitus soon after steroid therapy. At the time of rebiopsy, a high level of serum IgG4 was detected. The second renal biopsy showed diabetic nephropathy without any tubulointerstitial damage. The first biopsied tissues were retrospectively investigated. Large numbers of IgG4-positive plasma cells were detected in the kidneys and lymph nodes. A retrospective diagnosis of IgG4-related TIN with lymph node involvement was made. In conclusion, this paper describes a retrospectively diagnosed case of IgG4-related TIN with lymph node involvement, showing good clinical and pathological prognosis.

scholarly journals Giant Fibroadenoma in a Middle-Aged Woman Requiring Mastectomy: A Case Report

2020 ◽  
Vol 6 (4) ◽  
pp. 456-462
Author(s):  
NJ Nwashilli ◽  
I Obahiagbon
Keyword(s):  
Lymph Node ◽  
Axillary Lymph Node ◽  
Breast Tumour ◽  
Lymph Node Excision ◽  
Normal Breast ◽  
Main Concern ◽  
Axillary Lymph ◽  
Aged Woman ◽  
Large Breast ◽  
Operative Specimen

Fibroadenomas are benign tumours of the breast. They are usually single, firm, rubbery masses, slow-growing and well encapsulated. Giant fibroadenomas are fibroadenomas at least 5cm in size or at least 500g in weight. The peculiarities of the index case include the massive size and weight of the breast, causing asymmetry and tissue distortion with little or no normal breast tissue on ultrasound scan. Also, such massive weight has not been reported in the literature as suggested by extensive search on databases such as Pubmed and Google Scholar. The main concern of the patient was the rapid growth over a year, with the attendant risk of malignancy. The mass was firm, lobulated, with a solitary axillary lymph node. An initial clinical diagnosis of phyllodes tumour was made. However, pre-operative Tru-cut biopsy histology suggested fibroadenoma and was confirmed using the excised post-operative specimen. Simple mastectomy with axillary lymph node excision was carried out. In conclusion, a large breast tumour may not be malignant. However, mastectomy may be a treatment option despite the benign nature of the tumour. 

PERIPHERAL LYMPH NODE EXCISIONAL BIOPSY IN CHILDREN: YIELD IN A TERTIARY CARE CENTRE

2021 ◽  
pp. 67-70
Author(s):  
Dhvani Shah ◽  
Vishesh Dikshit ◽  
Apoorva Kulkarni ◽  
Abhaya Gupta ◽  
Paras Kothari ◽  
...  
Keyword(s):  
Lymph Node ◽  
Diagnostic Yield ◽  
Tertiary Care ◽  
Lymph Node Excision ◽  
Limited Resources ◽  
Excision Biopsy ◽  
Age Group ◽  
Peripheral Lymph Node ◽  
Peripheral Lymph ◽  
High Prevalence

AIM: To study the pediatric patients in a developing country undergoing peripheral lymph node excision biopsy in terms of demographics and histopathological ndings and evaluate the diagnostic yield of peripheral Lymph node excision biopsy in children. MATERIALS AND METHODS: A retrospective study of 402 patients was done of the children undergoing peripheral lymph node st st excision biopsy in a tertiary care center from 1 January 2013 to 31 December 2018 (6 years). Demographics, histopathological ndings and yields were studied. RESULTS: Out of the 402 patients, 218 (54.2%) were males and 184(45.8%) were females. Male to female ratio 1.18: 1. Maximum patients belonged to the age group of 4-6years (26.87%) followed by the age group of 2-4years of age (22.89%). Most common group of nodes excised were cervical (77.9%) followed by axillary and inguinal lymph nodes (9.2%) each. The most common etiology was reactive lymphoid hyperplasia (63.18%) in our study followed by tuberculous lymphadenitis(31.84%). Specic ndings were seen in 35.58% and non-specic ndings were seen in 64.42% patients. Malignancy was seen in 8 patients (2%). CONCLUSION: Peripheral Lymphadenopathy in pediatric population is always a diagnostic challenge. While FNAC tends to be investigation of choice for adults, in a developing country with limited resources and high prevalence of tuberculosis, peripheral lymph node biopsy is the gold standard with a good diagnostic yield and should always be considered for patients with persistent lymphadenopathy. Clinical significance: The study highlights the importance of excision biopsy in peripheral lymphadenopathy in children in a developing nation with limited resources and high prevalence of infectious diseases.

scholarly journals Multiple surgical radical treatment in axillary lymph nodes: A case report

2019 ◽  
Vol 17 ◽  
pp. 205873921983895
Author(s):  
Xiang Gao ◽  
Tang-Shun Wang ◽  
Juan Cheng ◽  
Xiao-Guang Shi ◽  
Ke-Xin Zhou ◽  
...  
Keyword(s):  
Lymph Node ◽  
Axillary Lymph Node ◽  
Regional Lymph Node ◽  
Radical Resection ◽  
Lymph Node Excision ◽  
Axillary Lymph Nodes ◽  
Axillary Lymph ◽  
Bacterial Infectious Disease ◽  
New Ideas ◽  
Lymph Node Tuberculosis

Lymph node tuberculosis is a common clinical bacterial infectious disease. Regional lymph node tuberculosis is often difficult to cure by surgically radical resection. In addition, its recurrence rate is higher, and it can easily cause lymphatic leakage. This case was considered to have left axillary lymph node tuberculosis. A combination of clinical examination, ultrasound, and magnetic resonance imaging examinations were performed before surgery. The surgical procedure performed was left axillary lymph node excision. Postoperative pathology confirmed the lymph node tuberculosis. The patient was given anti-tuberculosis drug treatment with no recurrence after 6 months follow-up. This provides new ideas and methods for the clinical treatment of regional lymph node tuberculosis.

scholarly journals Significance of axillary lymph node extranodal soft tissue extension and indications for postmastectomy irradiation

Cancer ◽  
1999 ◽  
Vol 86 (7) ◽  
pp. 1258-1262 ◽  
Author(s):  
John E. Mignano ◽  
Marianna L. Zahurak ◽  
Anuradha Chakravarthy ◽  
Steven Piantadosi ◽  
William C. Dooley ◽  
...  
Keyword(s):  
Lymph Node ◽  
Soft Tissue ◽  
Axillary Lymph Node ◽  
Axillary Lymph ◽  
Soft Tissue Extension

IgG4-Related Lymphadenopathy: a Distinct Clinicopathological Entity That Is Associated with IgG4-Related Sclerosing Diseases

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4910-4910
Author(s):  
Yayoi Fujiwara ◽  
Kenji Notohara ◽  
Yasuyuki Arai ◽  
Takahito Kawata ◽  
Takeshi Ito ◽  
...  
Keyword(s):  
Lymph Node ◽  
Serum Level ◽  
Lymph Nodes ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Serum Levels ◽  
Follicular Hyperplasia ◽  
Pet Ct ◽  
Plasma Cell Type ◽  
Serum Igg4

Abstract Background: Autoimmune pancreatitis (AIP) and its related diseases, such as sclerosing cholangitis, sclerosing sialadenitis, interstitial nephritis and retroperitoneal fibrosis, share IgG4-related abnormalities including elevated serum IgG4 level and numerous IgG4- positive plasma cells in the affected tissues. All of these diseases are now wrapped into a new clinicopathological entity, and is designated as IgG4-related sclerosing diseases (IgG4-SD). Lymphadenopathy is not uncommon in patients with IgG4-SD, but so far as we know, there has been only one study that describes the clinicopathological features of IgG4-related lymphadenopathy (IgG4-LN). In order to clarify whether IgG4-LN is a distinct and clinically significant entity, we reviewed lymph node biopsies which had been pathologically diagnosed as reactive or inflammatory. We also reviewed patients with IgG4-SD who had had CT and/or PET/CT scans to see if the extent of lymphadenopathy affects serum levels of IgG and IgG4. Design: The study protocol consists of two parts. The first part is a clinicopathological review of 713 lymph node biopsies which had been diagnosed as reactive or inflammatory. In our preliminary study with enlarged lymph nodes seen around pancreata with AIP or submaxillary glands with sclerosing sialadenitis, we had found that follicular hyperplasia and plasmacytic hyperplasia were characteristic. Thus we selected 87 lymph nodes with predominantly follicular and/or plasmacytic hyperplasia for the immunostains for IgG1 and IgG4. Lymph nodes were diagnosed to be IgG4-LA if IgG4-positive plasma cells were numerous (more than 10/high power field in average), and the number of IgG4- positive plasma cells was larger than or was comparable to that of IgG1-positive plasma cells. The second part of the study is a clinical review of 27 patients with IgG4-SD who had CT and/or PET/CT scans. They consisted of 15 cases with AIP, 4 with sclerosing sialadenitis, 2 with interstitial pneumonia, 6 with IgG4-LA (the cases included in the first part). By reviewing CT and/or PET/CT scan images, the number of regions with lymphadenopathy was evaluated according to the Ann Arbor stage, and was compared with serum levels of IgG and IgG4. Results: 11 cases fulfilled the criteria of IgG4-LA. The average age of the patients was 65.8 (range, 48–77) years; 9 were male. Three cases each were initially diagnosed as plasma cell type of Castleman’s disease and florid follicular hyperplasia; the rests were biopsied after we had recognized IgG4-LA. After the initial workup or in the followup, 5 cases were found to have other IgG4-SD, such as AIP, sclerosing sialadentitis, interstitial pneumonia, interstitial nephritis and retroperitoneal fibrosis. The serum level of IgG and IgG4 were 1366–6534 mg/dl (average, 4405) and 219–2750 mg/dl (average, 1363), respectively. Among the 27 cases in the second part of the study, the serum level of IgG and IgG4 were 1366–7953 mg/dl (average, 3243) and 122–4140 mg/dl (average, 1043), respectively. CT and/or PET/CT scan depicted lymphadenopathy in every case. The number of regions with lymphadenopathy was 3.43 in average (range, 1–8). The serum levels of both IgG and IgG4 significantly correlated with the number of regions with lymphadenopathy. The existence of lymphadenopathy in the cervical, mediastinal, axillary, paraaortic, iliac, and inguinal areas also correlated with the serum level of IgG and IgG4. Conclusions: We suggest that IgG4-related lesion exists in the lymph node, and that IgG4-LA is a proper name for it. Our proposal is justified by the observation that patients with IgG4-LA revealed elevated serum IgG4 level and occasional coexistence of other IgG4-SD. IgG4-LA might be included previously in plasma cell type of Castleman’s disease and florid follicular hyperplasia. We found that the extent of lymphadenopathy in patients with IgG4-SD correlated with serum level of IgG and IgG4, suggesting that the extent of lymphadenopathy may indicate the disease activity of IgG4-SD.

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