Myelomatous Ascites and Pleural Effusion in Relapsed Multiple Myeloma

Extramedullary multiple myeloma is seen in advanced and aggressive disease and occurs due to plasma cell infiltration of sites other than the bone marrow. Myelomatous ascites or pleural effusion is seen in less than 1 % of cases and can be differentiated from infectious etiologies based on fluid cytology.

POS0187 THE PROGNOSTIC FACTORS IN PREDICTING RELAPSE FOR IGG4-RELATED DISEASE: A LONG-TERM STUDY BASED ON THE CLINICAL AND PATHOLOGICAL CHARACTERISTICS OF THE PATIENTS

Background:The relationship between the pathological findings and disease relapse has not been well established.Objectives:We aim to investigate the clinical and pathological manifestations in relation with disease relapse in IgG4-RD, as well as identify prognostic factors in predicting relapsed disease.Methods:This study enrolled 71 patients newly diagnosed with IgG4-RD between Jan 2011 and April 2020, all of whom had received pathological examinations. Their pathological manifestations and clinical data were collected. Multivariate Cox regression and AUC (area under curve) were used to identify predictors for relapsed disease and assess the predictive value of these predictors, respectively.Results:During a follow-up period of 26 (range, 6-123) months, 4.2% (3/71) patients died. The remaining 68 patients were all treated with glucocorticoids with or without immunosuppressor continuously. By the end of follow-up, 47 (69.1%) patients sustained clinical remission, and 21 (30.9%) patients suffered relapsed disease with a median relapse time at 10 (6-30) months. We found that IgG4 ≥ 6.5g/L (OR 1.52-11.06), IgG ≥ 20.8g/L (OR 1.11-7.23), IgG4-RD responder index (RI) ≥ 9 (OR 1.28-11.37), and more IgG4+ plasma cell infiltration (≥ 60 / HPF in visceral organs, or ≥ 200 / HPF in head and neck organs) (OR 1.79-22.41) were all independent predictive factors for disease relapse. A prognostic score was explored for predicting recurrence in IgG4-RD, including three predictive factors (IgG ≥ 20.8g/L, IgG4-RD RI ≥9, and more IgG4+ plasma cell infiltration). The three-year relapse rate for the patients with no, one, two, and three risk factors were 0%, 27.3%, 66.7%, and 100%, respectively.Conclusion:Patients’ earlier IgG4 ≥ 6.5g/L, IgG ≥ 20.8g/L, IgG4-RD RI ≥9, and more IgG4+ plasma cell infiltration independently predicted disease relapse. We explored a prognostic score for predicting recurrence in IgG4-RD include three predictive factors (IgG ≥ 20.8g/L, IgG4-RD RI ≥9, and more IgG4+ plasma cell infiltration), which might be used to evaluate the risk of recurrence in IgG4-RD.References:[1]DESHPANDE V, ZEN Y, CHAN J K, et al. Consensus statement on the pathology of IgG4-related disease[J]. Mod Pathol,2012,25(9): 1181-1192.[2]JENNETTE J C, FALK R J, BACON P A, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides[J]. Arthritis Rheum,2013,65(1): 1-11.[3]OKAZAKI K, UMEHARA H. Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan[J]. Int J Rheumatol,2012,2012: 357071.[4]SHIMOSEGAWA T, CHARI S T, FRULLONI L, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology[J]. Pancreas,2011,40(3): 352-358.[5]OHARA H, OKAZAKI K, TSUBOUCHI H, et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012[J]. J Hepatobiliary Pancreat Sci,2012,19(5): 536-542.[6]KAWANO M, SAEKI T, NAKASHIMA H, et al. Proposal for diagnostic criteria for IgG4-related kidney disease[J]. Clin Exp Nephrol,2011,15(5): 615-626.[7]GOTO H, TAKAHIRA M, AZUMI A. Diagnostic criteria for IgG4-related ophthalmic disease[J]. Jpn J Ophthalmol,2015,59(1): 1-7.[8]MATSUI S, YAMAMOTO H, MINAMOTO S, et al. Proposed diagnostic criteria for IgG4-related respiratory disease[J]. Respir Investig,2016,54(2): 130-132.[9]WEN ZHANG J H S. Management of IgG4-related disease[J]. Lancet Rheumatol,2019,1: e55-e65.[10]EBBO M, DANIEL L, PAVIC M, et al. IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry[J]. Medicine (Baltimore),2012,91(1): 49-56.Disclosure of Interests:None declared

Isolated middle mediastinal mass associated with immunoglobulin G4-related disease

Background Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ disorder predominantly occurring in middle-aged to elderly male patients characterized by multi-organ fibrosis, specific pathological findings of storiform fibrosis with IgG4-positive plasma cell infiltration, and elevated serum IgG4 level. We herein report a rare presentation of IgG4-RD forming an isolated mass in the middle mediastinum mimicking a mediastinal tumor and discuss the clinical significance of mediastinal IgG4-RD. Case presentation An 82-year-old male patient without any symptom was referred due to left middle mediastinal mass (3.8 × 2.4 cm). Because of suspected lymphoma, Castleman’s disease, and lymphangitis due to tuberculosis, we performed a thoracoscopic resection for diagnosis and treatment. The mass was yellowish white with well-encapsulated, and storiform fibrosis with plasma cell infiltration, and obliterative phlebitis were observed microscopically. Additional immunohistochemical stain revealed IgG4-RD. Other radiological findings and serological results did not show evidence of other organs being affected from IgG4-RD nor autoimmune diseases. He is now followed at outpatient clinic without additional treatment for over a year, and an enhanced computed tomography does not show any recurrence. Conclusion It was a rare presentation of IgG4-RD forming isolated middle mediastinal mass, which suggests that we might suspect IgG4-RD for undetermined mediastinal mass in case of middle to elderly male patient.

Immunoglobulin G4-associated autoimmune hepatitis with peripheral blood eosinophilia: a case report

Background Immunoglobulin G4 (IgG4) associated autoimmune hepatitis (AIH) has been recognized as a type of autoimmune disease that responds to corticosteroid. The diagnosis is based on elevation of the serum IgG4 level, abundance of IgG4 enhanced plasma cell infiltration in the portal region of the liver, and satisfaction of the criteria for “definite AIH” under the revised International Autoimmune Hepatitis Group (IAIHG) scoring system. However, the clinical course of the disease is unclear. Case presentation A 65-year-old man with jaundice and peripheral blood eosinophilia. His IAIHG and simplified score was compatible with definite AIH and his IgG4 level was elevated. Magnetic resonance imaging did not reveal abnormalities in the hepatobiliary system or pancreas. A liver biopsy revealed interface hepatitis with IgG4 positive plasma cell infiltration in the portal region, without evidence of bile duct injury. He responded to 4-week period of induction prednisolone therapy and had no recurring symptoms under maintenance therapy of 5 mg prednisolone during the 3-year follow up. Conclusions This was a rare case that demonstrated an association between IgG4 associated AIH and the presence of peripheral blood eosinophilia.

Lupus mastitis with predominant kappa-restricted plasma cell infiltration: report of a rare case

Lupus mastitis (LM) is a rare complication of systemic lupus erythematosus (SLE) or discoid lupus erythematosus (DLE). The clinical presentations of LM may mimic breast malignancy, and biopsy or excision is usually performed. Histologically, LM is featured by lymphoplasmacytic inflammation involving breast ducts, lobules, blood vessels and adipose tissue. Characteristic hyaline fat necrosis can be noted in most cases. Here, we reported a case of LM in an elderly female patient who presented with bilateral breast lesions. Histologically, the breast lesions showed prominent hyaline fat necrosis and predominantly plasmacytic inflammation involving breast ducts, vessels and fat lobules. Fibrinoid necrosis of vessels was also noted. The infiltrated plasma cells were Kappa light chain-restricted, but did not show the immunophenotypes for a plasma cell neoplasm. In addition, the patient developed Kappa-restricted plasma cell myeloma 2 years later. The patient was followed up for 8 years, and her breast lesion did not show recurrence. The patient’s unique clinicopathological presentations indicated a potential correlation between her LM and subsequently developed myeloma. It also indicated that the immunophenotypical characterization of infiltrated plasma cells in LM patients with predominant plasma cell infiltration may be important to rule out potential plasma cell neoplasms.

Plasma Cell Infiltration on Histopathological Samples of Chronic Bone and Joint Infections due to Cutibacterium acnes: A series of 21 Cases

Introduction: Histopathological definition of bone and joint infection (BJI) is based on Mirra's criterion (≥ 5 polymorphonuclears (PMNs) per field in 5 high power fields (HPFs)). However, this definition does not seem appropriate for chronic BJIs caused by slow-growing germs such as Cutibacterium acnes (C. acnes). The aim of this study was to confirm that Mirra's criterion is not adequate for diagnosis of BJIs due to C. acnes. The second objective was to determine if plasma cell infiltration could be useful for the diagnosis of chronic BJIs due to C. acnes.Methods: We retrospectively selected 25 consecutive patients from 2009 to 2013 with chronic BJIs due to C. acnes. Histological analysis was performed on the 21 cases with at least two C. acnes positive cultures. In addition of Mirra's criterion, the number of plasma cells (≥5 plasma cells/5 HPFs, defined as “CRIOAc Lyon's criterion”) was implemented in the histopathological analysis. Patients were defined as infected, if at least one of the two criteria were present.Results: According to Mirra's and CRIOAc Lyon's criteria, positive histopathology was observed in 12 (57.1%) and 15 (71.4%) cases respectively. Considering the 9 cases with negative Mirra's criterion, high plasma cell infiltration (≥5 plasma cells per field/5 HPFs) was observed in 5 cases (55.6%), and low plasma cells infiltration (2-5 plasma cells per field/5 HPFs) was observed in 4 other cases (44.4%).Conclusions: Adding CRIOAc Lyon's criterion to Mirra's criterion might restore some histopathological diagnosis of chronic BJIs due to C. acnes when a chronic BJI is clinically suspected.