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Clinical Lymphoma, Myeloma & Leukemia Jan 2022POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy/edema, monoclonal plasma protein [M protein], and skin changes) is a rare paraneoplastic disorder associated...
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy/edema, monoclonal plasma protein [M protein], and skin changes) is a rare paraneoplastic disorder associated with underlying plasma cell neoplasia. Although limited-stage disease can be treated with radiotherapy, treatment for the more advanced disease remains unclear. The most commonly used therapies for POEMS syndrome include alkylators and steroids, high-dose chemotherapy with autologos stem cell transplantation, lenalidomide, and bortezomib. In general, patients tend to have excellent prognosis if the diagnosis is made early and appropriate therapy is used. Here we present a systematic review of the efficacy and safety of treatment regimens used to treat POEMS syndrome in the adult population. Combinations of immunomodulatory agents with corticosteroids were most frequently utilized regimens with durable hematological and neurological responses. Combinations of proteasome inhibitors and alkylating agents with corticosteroids, although less frequently utilized, appear to have reasonable safety and efficacy profiles.
Topics: Female; Humans; Male; Middle Aged; POEMS Syndrome
PubMed: 34507924
DOI: 10.1016/j.clml.2021.07.033 -
Eye (London, England) Jul 2022Paraneoplastic syndromes affecting the visual system are a group of conditions that arise in the systemic malignancy framework. In this review, we have provided a... (Review)
Review
Paraneoplastic syndromes affecting the visual system are a group of conditions that arise in the systemic malignancy framework. In this review, we have provided a detailed and comprehensive overview of the published literature on the various ophthalmic paraneoplastic manifestations. A systematic review of many databases has been performed to identify ample literature on the paraneoplastic syndromes related to ophthalmology. We have discussed here the clinical features, pathogenesis, and treatment strategies of various ophthalmic paraneoplastic syndromes. It can be challenging to distinguish these disorders from their non-paraneoplastic counterparts and to determine the appropriate systemic assessment for the tumour responsible, to have a proper approach towards the management of the syndrome. METHOD: We searched PubMed, Science Direct and Journal of Ophthalmology for studies published in English between 1995 and April 2020, incorporating the general search term "paraneoplastic ocular syndrome" with connecting terms relevant to subheadings-e.g. Key search terms were cancer-associated retinopathy, (CAR), melanoma-associated retinopathy, (MAR), paraneoplastic retinopathy, autoimmune retinopathy, autoimmune-related retinopathy, and optic neuropathy, (ARRON), acute zonal occult outer retinopathy, (AZOOR), paraneoplastic vitelliform maculopathy, paraneoplastic vitelliform retinopathy, bilateral diffuse uveal melanocytic proliferation, (BDUMP), paraneoplastic optic neuropathy, (PON), polyneuropathy, organomegaly, endocrinopathy, monoclona gammopathy, and skin changes syndrome (POEMS) and various other terms. References from identified studies have been reviewed and included if deemed appropriate, valid, and scientifically important. If referenced in a selected English paper, we contemplated papers in other languages too. We preferentially selected papers that have been published in the last 10 years, but we have included relevant older references.
Topics: Autoantibodies; Autoimmune Diseases; Humans; Optic Nerve Diseases; Paraneoplastic Syndromes, Ocular; Retinal Diseases; Retinal Neoplasms
PubMed: 34345027
DOI: 10.1038/s41433-021-01676-x