Calciphylaxis in POEMS syndrome: Case report
Year: 2024
Type: article
Study design: Case Report
Abstract: POEMS Syndrome is a constellation of findings including Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, and Skin changes. Calciphylaxis, a microangiopathy involving vascular calcification and thrombotic occlusions, occurs rarely in POEMS. We present a case of prominent calciphylaxis that antedated the diagnosis of POEMS. The patient presented with extensive ecchymoses progressing to necrotic lesions in the setting of acute renal injury. Previously, she had chronic slowly progressive polyneuropathy, splenomegaly, hypothyroidism, amenorrhea, and ascites. Calciphylaxis was diagnosed on skin biopsy, and POEMS was diagnosed based upon clinical findings plus a bone marrow biopsy showing 15% lambda chain restricted plasma cells. Treatment for the calciphylaxis was supportive with fluids, tissue debridement, wound vacuum devices and antibiotics for secondary infection. Myeloma was treated with bortezomib and steroids. All aspects of the patient's manifestations improved. We conclude that calciphylaxis can be a prominent feature of POEMS and can appear prior to recognition of the full-blown syndrome.
Source: Rare
Institutions: National Institutes of Health, Office of the Director, NIH Common Fund, Fred Hutch Cancer Center, National Institutes of Health Clinical Center, National Cancer Institute, Center for Cancer Research, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Human Genome Research Institute
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $1,700
Funders: National Institutes of Health, Department of Laboratory Medicine and Pathology, University of Washington, National Human Genome Research Institute, NIH Office of the Director, National Cancer Institute, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH Clinical Center, Common Fund
Award: G5294359063
OpenAlex ID: W4391486851