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Clinical Journal of Gastroenterology

, Volume 9, Issue 4, pp 243–251 | Cite as

Systemic immunoglobulin light-chain amyloidosis presenting hematochezia as the initial symptom

  • Tetsuo Kon
  • Naoki NakagawaEmail author
  • Fumitsugu Yoshikawa
  • Kazunao Haba
  • Nagako Kitagawa
  • Michihiro Izumi
  • Setsuo Kumazaki
  • Satoshi Ishida
  • Ryuichi Aikawa
Case Report

Abstract

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.

Keywords

Immunoglobulin light-chain amyloidosis Kappa immunoglobulin light chain Submucosal hematoma Gastrointestinal bleeding 

Notes

Acknowledgments

We would like to thank Dr. Yuji Kozuka and the staff of the Kuwana West Medical Center clinical laboratory for the pathological studies.

Compliance with ethical standards

Conflict of interest

Tetsuo Kon, Naoki Nakagawa, Fumitsugu Yoshikawa, Kazunao Haba, Nagako Kitagawa, Michihiro Izumi, Setsuo Kumazaki, Satoshi Ishida, and Ryuichi Aikawa declare that they have no conflict of interest.

Human/animal Rights

All procedures followed have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

Informed consent was obtained from all patients for being included in the study.

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Copyright information

© Japanese Society of Gastroenterology 2016

Authors and Affiliations

  • Tetsuo Kon
    • 1
  • Naoki Nakagawa
    • 1
    Email author
  • Fumitsugu Yoshikawa
    • 1
    • 2
  • Kazunao Haba
    • 1
  • Nagako Kitagawa
    • 1
  • Michihiro Izumi
    • 1
  • Setsuo Kumazaki
    • 1
  • Satoshi Ishida
    • 1
  • Ryuichi Aikawa
    • 1
    • 2
  1. 1.Department of Internal MedicineKuwana West Medical CenterKuwanaJapan
  2. 2.Department of Internal MedicineKuwana South Medical CenterKuwanaJapan

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