Laminar crystal deposition in large vessels in a patient with crystalglobulinemia
Laminar crystal deposition in large vessels in a patient with crystalglobulinemia
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DOI：
10.1111/pin.12526
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发表时间：
2017-05-01
影响因子：
2.2
通讯作者：
Fukayama, Masashi
中科院分区：
文献类型：
--
作者：
Shintani-Domoto, Yukako;Shinozaki-Ushiku, Aya;Fukayama, Masashi
结晶球蛋白血症是一种极其罕见的单克隆性丙种球蛋白病的并发症，其特征是全身器官内有结晶血栓。我们在此报告第一例结晶性球蛋白血症，并伴有大血管内的片状晶体沉积。患者男，44岁，除视力受损外，还伴有左腿麻木、疼痛和肿胀的病史。肾脏和皮肤活检发现毛细血管管腔内有结晶血栓。患者最终被诊断为与多发性骨髓瘤相关的结晶球蛋白血症。他接受了血液透析和化疗，但在入院15个月后死于这种疾病。尸检显示胸主动脉至腹主动脉血管壁内膜下有大量结晶沉积。特征性沉积累及髂动脉、颈总动脉和锁骨下动脉，但不影响双侧肾动脉。死前CT显示腹主动脉壁高信号，而肾动脉壁未见高信号，提示CT高信号可作为全身晶体沉积的临床标志。
Crystalglobulinemia is an extremely rare complication of monoclonal gammopathy and is characterized by crystal thrombi within systemic organs. We herein report the first described case of crystalglobulinemia accompanied by laminar crystal deposition in the large vessels. A 44-year-old man presented with a history of numbness, pain, and swelling of the left leg in addition to visual impairment. Renal and skin biopsies revealed crystal thrombi within the capillary lumens. The patient was finally diagnosed with crystalglobulinemia associated with multiple myeloma. He was treated with hemodialysis and chemotherapy but died of the disease 15 months after admission. Autopsy demonstrated a huge amount of crystal deposition in the subintimal layer of the vascular wall throughout the thoracic to abdominal aorta. The characteristic deposition extended to the iliac arteries, common carotid arteries, and subclavian arteries but did not affect the bilateral renal arteries. Antemortem computed tomography demonstrated higher intensity in the wall of the abdominal aorta but not in the walls of the renal arteries, suggesting that a finding of high intensity on computed tomography could be a clinical marker of systemic crystal deposition.