POEMS综合症是包括多发性神经病在内的一系列发现,器官肿大,内分泌疾病,单克隆浆细胞疾病,皮肤的变化。钙化病,涉及血管钙化和血栓性闭塞的微血管病,很少发生在POEMS中。我们介绍了一例突出的钙化病,该病例早于POEMS的诊断。在急性肾损伤的情况下,患者出现广泛的瘀斑,进展为坏死性病变。以前,她患有慢性缓慢进行性多发性神经病,脾肿大,甲状腺功能减退,闭经,和腹水。皮肤活检诊断为钙化病,根据临床表现和骨髓活检显示15%的λ链限制性浆细胞诊断POEMS。钙化的治疗是用液体支持,组织清创术,伤口真空设备和抗生素继发感染。骨髓瘤用硼替佐米和类固醇治疗。患者的所有方面的表现都得到了改善。我们得出的结论是,钙化可能是POEMS的突出特征,并且可以在认识到全面综合征之前出现。
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.