Systemic immunoglobulin light-chain amyloidosis presenting hematochezia as the initial symptom.
Immunoglobulin light-chain (AL) amyloidosis is characterized by the deposition of insoluble fibrils composed of immunoglobulin light chains secreted by monoclonal plasma cells. Given the recent advances in the therapy of AL amyloidosis, it is important to diagnose this disease as early as possible. Herein, we describe the case of a 62-year-old man with hepatitis C virus (HCV)-related cirrhosis presenting with hematochezia. Colonoscopy showed multiple submucosal hematomas within the region ranging from the transverse colon to the sigmoid colon. Kappa immunoglobulin light-chain amyloid deposition was also detected. Bone marrow examination revealed a monoclonal abnormal plasma cell population. Thus, the patient was diagnosed with systemic immunoglobulin light-chain amyloidosis. The hematochezia was conservatively managed. However, because of liver failure caused by liver cirrhosis, the patient developed massive pleural effusion and died of respiratory failure. Postmortem examination revealed amyloid deposition in the esophagus, stomach, duodenum, ileum, descending colon, pancreas, heart, and lung. In these organs, amyloid deposition was limited to the vascular wall. We concluded that AL amyloidosis can present hematochezia arising from submucosal hematoma in the large colon before other systemic symptoms appear.
Department of Internal Medicine, Kuwana West Medical Center, 416-1 Kitabessho, Kuwana, Mie, 511-0819, Japan.
Tetsuo
T
Kon
Department of Internal Medicine, Kuwana West Medical Center, 416-1 Kitabessho, Kuwana, Mie, 511-0819, Japan. n-nakagawa@kuwanacmc.or.jp.
Naoki
N
Nakagawa
Department of Internal Medicine, Kuwana West Medical Center, 416-1 Kitabessho, Kuwana, Mie, 511-0819, Japan.|Department of Internal Medicine, Kuwana South Medical Center, 1 Chome-32-1 Chuocho, Kuwana, Mie, 511-0068, Japan.
Fumitsugu
F
Yoshikawa
Department of Internal Medicine, Kuwana West Medical Center, 416-1 Kitabessho, Kuwana, Mie, 511-0819, Japan.
Kazunao
K
Haba
Department of Internal Medicine, Kuwana West Medical Center, 416-1 Kitabessho, Kuwana, Mie, 511-0819, Japan.
Nagako
N
Kitagawa
Department of Internal Medicine, Kuwana West Medical Center, 416-1 Kitabessho, Kuwana, Mie, 511-0819, Japan.
Michihiro
M
Izumi
Department of Internal Medicine, Kuwana West Medical Center, 416-1 Kitabessho, Kuwana, Mie, 511-0819, Japan.
Setsuo
S
Kumazaki
Department of Internal Medicine, Kuwana West Medical Center, 416-1 Kitabessho, Kuwana, Mie, 511-0819, Japan.
Satoshi
S
Ishida
Department of Internal Medicine, Kuwana West Medical Center, 416-1 Kitabessho, Kuwana, Mie, 511-0819, Japan.|Department of Internal Medicine, Kuwana South Medical Center, 1 Chome-32-1 Chuocho, Kuwana, Mie, 511-0068, Japan.
Ryuichi
R
Aikawa
D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis; D001706:Biopsy; D003106:Colon / Q000473:pathology; D003108:Colonic Diseases / Q000209:etiology; D003113:Colonoscopy; D017809:Fatal Outcome; D006471:Gastrointestinal Hemorrhage / Q000209:etiology*; D006406:Hematoma / Q000209:etiology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis*; D008099:Liver / Q000473:pathology; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed
D007147:Immunoglobulin Light Chains
Gastrointestinal bleeding; Immunoglobulin light-chain amyloidosis; Kappa immunoglobulin light chain; Submucosal hematoma