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Metabolic disorders and reticulohistiocytic proliferative disorders
Published in Rashmi Sarkar, Anupam Das, Sumit Sethi, Concise Dermatology, 2021
Idiopathic disorders are characterized clinically by a generalized papular eruption and induration of skin (sclerodermoid appearance) and histopathologically by the triad of mucin deposition, increased fibroblast proliferation, and fibrosis. The exact pathogenesis is unknown; it may be associated with a monoclonal gammopathy and has systemic implications. The age of onset is between 30 and 80 years. There is no ethnic or sexual predilection. Scleromyxedema should be differentiated from other localized forms of lichen myxedematosus, where the skin is the sole site of involvement. Treatment is usually targeted against the gammopathy and can often be disappointing.
Diseases of the Peripheral Nerve and Mononeuropathies
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Diana Mnatsakanova, Charles K. Abrams
Other etiologies for neuropathy, although less likely, should be ruled out. These include: Alcohol use.Vitamin deficiencies.Toxic/drug-induced.Monoclonal gammopathy.Renal or hepatic insufficiency.
The lymphoreticular system and bone marrow
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Some patients presenting with a low level of serum paraprotein are found to have <10% monoclonal plasma cells in the bone marrow. These patients display none of the clinical features of myeloma and lack bony lesions. This condition is called monoclonal gammopathy of undetermined significance (MGUS) and has a high prevalence (3%) after the age of 70 years. Most patients require no treatment, although plasma cell myeloma or amyloidosis will develop in 25% of cases.
Factors affecting the accuracy of amyloidosis identification and referral to a specialty centre
Published in Amyloid, 2023
Andrew Staron, Lisa M. Mendelson, Tracy Joshi, Frederick L. Ruberg, Vaishali Sanchorawala
The retrospective design of this study leaves some gaps in our understanding of the implications of inaccurate diagnostic results for patients undergoing workup for amyloidosis. Our cohort consisted solely of individuals referred by outside providers for evaluation. Thus, the investigation could not provide information about false negative results (i.e. patients who should have been referred for amyloidosis but were not due to a negative result). The lack of prospective follow-up of false positive referrals prevented us from being certain that amyloidosis was not diagnosed at a later time point. On a final thought, the referrals described in this study—although false—were not necessarily inappropriate. A handful of people were able to discontinue plasma cell-directed chemotherapy for a monoclonal gammopathy without amyloidosis; many more were likely spared inappropriate exposure to treatments and related toxicities by referral to our centre. The time and resources spent on false positive referrals need to be weighed against the value of education and reassurance gained by patients and community-based healthcare providers. Multidisciplinary collaborations at centres of excellence for amyloidosis can offer a shared and accurate understanding of disease status.
Enhancing prognostication and personalizing treatment of extranodal marginal zone lymphoma
Published in Expert Review of Hematology, 2023
Juan Pablo Alderuccio, Izidore S. Lossos
Analyzing the University of Miami EMZL dataset, we found shorter PFS (HR = 1.82, P = 0.016) and OS (HR = 2.25, P = 0.033) in patients presenting with monoclonal gammopathy (MG) at diagnosis [58]. MG was present in 10.7% of the patients and was characterized by predominance of IgG as the most common paraprotein. In other series, IgM has been described as the most common MG in EMZL, but this difference could be secondary to difference in EMZL disease location, analysis of small cohorts, and possible inclusion of patients with lymphoplasmacytic lymphomas which occasionally may be difficult to differentiate from EMZL [63,64]. We also observed a higher incidence of MG in patients with gastrointestinal (GI)-non gastric (12.5%) and multiple mucosal sites (MMS) (11.6%) presentations. Other studies support this observation, associating the presence of MG at diagnosis with worse outcome in EMZL [65].
Acute kidney injury due to thrombotic microangiopathy in a patient with primary Sjögren’s syndrome
Published in Renal Failure, 2022
Yi Wang, Xun Zhou, Xiaoyan Ma, Xinyu Yang, Yishu Wang, Min Tao, Binbin Cui, Tianyu Xiao, Shougang Zhuang, Na Liu
The standard treatment of TMA associated with monoclonal gammopathy remains unknown. With the goal of eradicating the clonal cells producing the immunoglobulin, bortezomib-based regimens for a non-IgM monoclonal immunoglobulin was recommended [8]. Clone-targeted chemotherapy might improve clinical parameters in monoclonal gammopathy-associated TMA was currently reported. Cheungpasitporn et al. reported a case of successful treatment with bortezomib, lenalidomide, and dexamethasone (VRD) regimen for a patient with aHUS and monoclonal protein refractory to eculizumab therapy [9]. The patient had a significant improvement in proteinuria, renal function and MAHA since starting VRD. The efficacy of bortezomib in the present case confirmed the possible mechanism that monoclonal gammopathy may be associated with TMA via complement dysregulation.