Pilot study of Ixazomib, Lenalidomide, and Dexamethasone for Patients with POEMS Syndrome
POEMS syndrome is a rare paraneoplastic syndrome caused by an underlying plasma cell disorder. The combination of a proteasome inhibitor, an IMiD and corticosteroid is known to be highly effective among patients with myeloma.
In this study we sought to identify mechanisms of disease development by comparing the immune tumor microenvironment (iTME) of patients with POEMS syndrome to that of patients with MGUS.
ConclusionThis retrospective analysis provides important information on the clinical characteristics of POEMS syndrome in Indian patients, which will help the clinician’s decision-making process.
This study is aimed at bringing together various evidences from the field of immunological and hematological research, at exposing contradictions, and at revealing novel insights on the association between ANCA-associated vasculitis and hematologic malignancies. PMID: 31198793 [PubMed - in process]
ConclusionThe data presented here would be key to the clinician’s decision-making process particularly in the Indian context.
We report a retrospective analysis of 49 cases from a single tertiary-care center in New Delhi, India, including 10 patients who underwent hematopoietic stem-cell transplantation. Clinical features, responses, and outcomes were similar to the Western and Eastern literature, though with some unique features.
Multiple myeloma is a malignancy of the plasma cell usually associated with a monoclonal gammopathy, most commonly IgG and IgA.1 While the classic symptoms of multiple myeloma are related to hypercalcemia, renal failure, anemia, and bone disease,1,2 multiple myeloma may also be associated with cutaneous findings, including direct infiltration of malignant cells into the skin presenting as cutaneous plasmacytoma,3 deposition of paraproteins leading to nasal and facial spicules,4 leukocytoclastic vasculitis,5 cryoglobulinemia, amyloidosis, xanthomas,6 hemangiomas, hyperpigmentation and hypertrichosis as seen in POEMS syndrom...
We report a case of PNP successfully treated with pulsing doses of steroids, mycophenolate and rituximab-bendamustine with complete negativization of epidermal-proteins auto-immune antibodies.
POEMS syndrome is a rare paraneoplastic syndrome associated with clonal plasma cells. ASCT is believed to be an effective treatment in the control of the disease. Patient-, disease-&transplant-related variables were collected from the EBMT database. Systemic involvement &response was detailed utilizing an organ response tool pre- &post-ASCT. 127 patients underwent an ASCT between 1997-2010. The median age was 49.9 years (range 26.3, 69 years). The median time from diagnosis to ASCT was 7.5 months (range 0.8, 346).
We report the clinical features, treatment and outcomes of a retrospective series of 43 cases from our experience at a tertiary care centre in New Delhi, India including 6 patients who received ASCT.