title: Laminar crystal deposition in large vessels in a patient with crystalglobulinemia.
abstract: 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.
authors.affiliation: Department of Pathology and Diagnostic Pathology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, Japan.
authors.forename: Yukako
authors.initials: Y
authors.lastname: Shintani-Domoto
authors.affiliation: Department of Pathology and Diagnostic Pathology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, Japan.
authors.forename: Aya
authors.initials: A
authors.lastname: Shinozaki-Ushiku
authors.affiliation: Department of Radiology, The University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan.
authors.forename: Hidemi
authors.initials: H
authors.lastname: Okuma
authors.affiliation: Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan.
authors.forename: Mineo
authors.initials: M
authors.lastname: Kurokawa
authors.affiliation: Department of Pathology and Diagnostic Pathology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Fukayama
mesh_terms: D000328:Adult; D001012:Aorta, Abdominal / Q000000981:diagnostic imaging / Q000473:pathology; D001344:Autopsy; D001706:Biopsy; D003449:Cryoglobulinemia / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D003450:Cryoglobulins / Q000378:metabolism; D017809:Fatal Outcome; D006801:Humans; D007083:Iliac Artery / Q000000981:diagnostic imaging / Q000473:pathology; D007668:Kidney / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D009101:Multiple Myeloma / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D010265:Paraproteinemias / Q000150:complications* / Q000628:therapy; D012867:Skin / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed
chemical_list: D003450:Cryoglobulins
keywords: cryoglobulins; crystallization; monoclonal gammopathy