Novacic Danica, Uldrick Thomas, Dulau-Florea Alina, Howe Colleen Evans, Lee Chyi-Chia R, Kong Heidi H, Gahl William A
Undiagnosed Diseases Program, National Institutes of Health (NIH) Common Fund, Office of the Director, NIH, Bethesda, MD, USA.
Vaccine and Infectious Disease Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA.
Rare. 2024;2. doi: 10.1016/j.rare.2024.100019. Epub 2024 Feb 1.
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.
POEMS综合征是一组临床表现，包括多发性神经病、器官肿大、内分泌病、单克隆浆细胞病和皮肤改变。钙化防御是一种涉及血管钙化和血栓闭塞的微血管病，在POEMS综合征中很少发生。我们报告一例在POEMS综合征诊断之前就已出现明显钙化防御的病例。该患者在急性肾损伤的情况下出现广泛瘀斑并进展为坏死性病变。此前，她患有慢性缓慢进展性多发性神经病、脾肿大、甲状腺功能减退、闭经和腹水。皮肤活检诊断为钙化防御，根据临床表现及骨髓活检显示15%的λ链限制性浆细胞诊断为POEMS综合征。钙化防御的治疗包括补液、组织清创、伤口负压装置以及针对继发感染使用抗生素等支持治疗。骨髓瘤采用硼替佐米和类固醇治疗。患者所有的临床表现均有改善。我们得出结论，钙化防御可以是POEMS综合征的一个突出特征，并且可能在完全型综合征被识别之前就出现。
