Monoclonal gammopathy of clinical significance : an IgG-lambda monoclonal gammopathy associated with renal failure, lung disease and cutis laxa

Author(s):  
Laure Maindiaux
2019 ◽  
Vol 19 (10) ◽  
pp. e334
Author(s):  
Pauline Storms ◽  
Géraldine Verstraete ◽  
Valentine Gillion ◽  
Eric Goffin ◽  
Selda Aydin ◽  
...  

2009 ◽  
Vol 36 (1) ◽  
pp. 115-117 ◽  
Author(s):  
Paolo Gavarotti ◽  
Felice Fortina ◽  
Daniela Costa ◽  
Giuseppe Verzetti ◽  
Valter Redoglia ◽  
...  

1998 ◽  
Vol 60 (7) ◽  
pp. 805-808 ◽  
Author(s):  
Mika MISHINA ◽  
Toshifumi WATANABE ◽  
Kouichi FUJII ◽  
Hiroto MAEDA ◽  
Yoshito WAKAO ◽  
...  

2020 ◽  
Vol 2020 ◽  
pp. 1-10
Author(s):  
Sophia Z. Shalhout ◽  
Myrna R. Nahas ◽  
Reed E. Drews ◽  
David M. Miller

Background. Cutis laxa is a rare dermatosis that is inherited or acquired and clinically features loose, wrinkled, and redundant skin with decreased elasticity. This heterogeneous connective tissue disorder may be localized or generalized, with or without internal manifestations. Generalized cutis laxa often has a cephalocaudal progression and is attributed to inflammatory cutaneous eruptions, medications, and infections. Cutis laxa is also associated with several other conditions including rheumatoid arthritis, systemic lupus erythematosus, and plasma-cell dyscrasias. Case Presentation. We report an unusual case of a 35-year-old male with progression of generalized acquired cutis laxa and vasculitis that occurred over a period of one year. No cutaneous inflammatory eruption preceded or accompanied his decreased skin elasticity, and a biopsy of the skin showed elastolysis. His cutaneous manifestation led to systemic evaluation and an eventual diagnosis of smoldering multiple myeloma accompanied by aortitis and anemia. His myeloma and vasculitis were successfully treated with cyclophosphamide, bortezomib, and dexamethasone and high-dose prednisone, respectively, with no improvement to his cutis laxa. Conclusions. The presence of monoclonal gammopathy is strongly associated with several dermatological entities such as acquired cutis laxa. We propose a new term for the dermatological manifestations caused by paraproteinemia: monoclonal gammopathy of dermatological significance, or MGODS, and stress the evaluation of an underlying gammopathy in the setting of certain dermatologic conditions, including scleromyxedema and amyloidosis. We present a case of a newly acquired cutis laxa secondary to plasma-cell dyscrasias that exemplifies MGODS, alongside a brief literature review, and underscore the clinical relevance of monoclonal gammopathies of dermatological significance.


Hematology ◽  
2020 ◽  
Vol 2020 (1) ◽  
pp. 380-388
Author(s):  
Angela Dispenzieri

Abstract “Monoclonal gammopathy of clinical significance” (MGCS) is the term used to describe nonmalignant monoclonal gammopathies causing important disease. MGCS is the differential diagnosis for any patient presenting with what appears to be a monoclonal gammopathy of undetermined significance but is also experiencing other unexplained symptoms. Broadly, these conditions can be separated into symptoms and signs referable to the nerves, the kidneys, and the skin. The first step in making these diagnoses is to consider them. With a particular condition in mind, the next step is to order those tests that can help confirm or dismiss a particular diagnosis. Nearly all of the renal and dermatologic conditions are diagnosed by renal and skin biopsies, respectively. The importance of a highly competent renal pathologist and dermatopathologist cannot be underestimated. Biopsy is less specific for the neuropathic conditions. Because several of the MGCSs are syndromes, recognizing other manifestations is also key. Treatment recommendations for many of these conditions are anecdotal because of their rarity, but for several of the conditions, IV immunoglobulin, rituximab, and plasma cell–directed therapy are the best options.


2018 ◽  
Vol 67 (4) ◽  
pp. 761-766
Author(s):  
Li Wei ◽  
Jing Zhao ◽  
Jing Bao ◽  
Yanliang Ma ◽  
Ying Shang ◽  
...  

To investigate the expression and clinical significance of secretory mucins in patients with interstitial lung disease (ILD). The bronchoalveolar lavage fluid (BALF) concentrations of mucins (MUCs) from 27 patients with ILD, 6 patients with lung cancer, 8 patients with pleural effusion and 9 patients with bronchiectasis were determined by ELISA. The concentration of MUC5AC was significantly increased in patients with ILD (12.84±15.02 ng/mL) compared with patients with pleural effusion (4.33±2.51 ng/mL), lung cancer (8.02±5.57 ng/mL) or bronchiectasis (6.08±2.40 ng/mL) (p<0.01). The MUC2 level (10.23±9.27 ng/mL) was significantly elevated in patients with ILD than in those with pleural effusion (6.21±3.28 ng/mL) or bronchiectasis (5.73±1.51 ng/mL) (both p<0.05). Patients with ILD (104.64±61.61 ng/mL), lung cancer (148.45±169.24 ng/mL) or bronchiectasis (123.68±63.28 ng/mL) had significantly greater IL-8 levels than in those with pleural effusion (76.46±2.16 ng/mL) (p<0.05). A significant positive correlation was detected between the MUC5AC concentration and the lymphocyte percentage in BALF of patients with ILD (r=0.504, p=0.007). Lung function tests of patients with ILD exhibited various degrees of restrictive ventilation dysfunction and reduced diffusing capacity. The MUC5AC levels in BALF were negatively correlated with forced expiratory volume in 1 second (FEV1)/forced vital capacity (r=−0.761, p=0.000), FEV1 predicted value (FEV1/pred) (r=−0.668, p=0.002), and diffusing capacity (r=−0.606, p=0.006). Secretory mucins MUC5AC, MUC2 and IL-8 were highly expressed in ILD. MUC5AC level was closely correlated with the amount of inflammatory cells in BALF and the lung function parameters.


2018 ◽  
Vol 79 (5) ◽  
pp. 945-947 ◽  
Author(s):  
Marie Jachiet ◽  
Stéphanie Harel ◽  
Anne Saussine ◽  
Maxime Battistella ◽  
Michel Rybojad ◽  
...  

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