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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.
Multiple Myeloma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Multiple myeloma (MM) is a malignant disease of plasma cells in the bone marrow and forms part of a spectrum of plasma cell disorders that also includes monoclonal gammopathy of undetermined significance (MGUS), solitary plasmacytoma (bone or extramedullary), and AL amyloidosis. Myeloma is characterized by the production of a monoclonal immunoglobulin (Ig) molecule and serum free light chain and is associated with bone destruction, anemia, infection, and renal failure. There has been a significant improvement in survival in the last two decades with the use of proteasome inhibitors, immunomodulatory drugs, and monoclonal antibodies, but still only a minority of patients survive beyond 10 years. New antibodies (anti-BCMA for example), new drugs (venetoclax, selinexor), and immunomodulatory approaches will further improve outcomes.
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.
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.
Zanubrutinib for the treatment of adults with Waldenstrom macroglobulinemia
Published in Expert Review of Anticancer Therapy, 2022
Shayna Sarosiek, David Sermer, Andrew R. Branagan, Steven P. Treon, Jorge J. Castillo
Waldenström macroglobulinemia (WM) is an indolent B-cell lymphoma characterized by the presence of an IgM monoclonal gammopathy and a bone marrow infiltrate of clonal lymphoplasmacytic cells [1]. Whole genome sequencing has revealed an activating mutation in MYD88, which is present in higher than 90% of patients with WM [2,3]. This mutation is the result of a substitution of leucine to proline in position 265 of MYD88 (MYD88L265P) and results in the downstream activation of nuclear factor-kappa B (NF-ĸB) through Bruton tyrosine kinase (BTK) and interleukin-1 receptor-associated kinases in the B-cell receptor pathway. Dysregulation of this pathway can lead to unregulated cell survival, proliferation, and migration and has been implicated in the development of many lymphoid disorders.