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


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.


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



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.


  1. 1.
    Dispenzieri A, Gertz MA, Buadi F. What do I need to know about immunoglobulin light chain (AL) amyloidosis? Blood Rev. 2012;26:137–54.CrossRefPubMedGoogle Scholar
  2. 2.
    Rosenzweig M, Landau H. Light chain (AL) amyloidosis: update on diagnosis and management. J Hematol Oncol. 2011;4:47.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Cohen AD, Comenzo RL. Systemic light-chain amyloidosis: advances in diagnosis, prognosis, and therapy. Hematol Am Soc Hematol Educ Program. 2010;2010:287–94.Google Scholar
  4. 4.
    Merlini G, Stone MJ. Dangerous small B-cell clones. Blood. 2006;108:2520–30.CrossRefPubMedGoogle Scholar
  5. 5.
    Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med. 1997;337:898–909.CrossRefPubMedGoogle Scholar
  6. 6.
    Matsuda M, Katoh N, Ikeda S. Clinical manifestations at diagnosis in Japanese patients with systemic AL amyloidosis: a retrospective study of 202 cases with a special attention to uncommon symptoms. Intern Med. 2014;53:403–12.CrossRefPubMedGoogle Scholar
  7. 7.
    Huang X, Wang Q, Jiang S, et al. The clinical features and outcomes of systemic AL amyloidosis: a cohort of 231 Chinese patients. Clin Kidney J. 2015;8:120–6.CrossRefPubMedGoogle Scholar
  8. 8.
    James DG, Zuckerman GR, Sayuk GS, et al. Clinical recognition of Al type amyloidosis of the luminal gastrointestinal tract. Clin Gastroenterol Hepatol. 2007;5:582–8.CrossRefPubMedGoogle Scholar
  9. 9.
    Klatt EC. Robbins and cotran atlas of pathology. 2nd ed. United States: Saunders; 2009.Google Scholar
  10. 10.
    Mumford A, O’Donnell J. Bleeding symptoms and coagulation abnormalities in 337 patients with AL-amyloidosis. Br J Haematol. 2000;110:454–60.CrossRefPubMedGoogle Scholar
  11. 11.
    Thompson CA, Kyle R, Gertz M, et al. Systemic AL amyloidosis with acquired factor X deficiency: a study of perioperative bleeding risk and treatment outcomes in 60 patients. Am J Hematol. 2010;85:171–3.PubMedPubMedCentralGoogle Scholar
  12. 12.
    Choufani EB, Sanchorawala V, Ernst T, et al. Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: incidence, bleeding manifestations, and response to high-dose chemotherapy. Blood. 2001;97:1885–7.CrossRefPubMedGoogle Scholar
  13. 13.
    Spier BJ, Einstein M, Johnson EA, et al. Amyloidosis presenting as lower gastrointestinal hemorrhage. WMJ. 2008;107:40–3.PubMedGoogle Scholar
  14. 14.
    Kim S-Y, Moon S-B, Lee SK, et al. Light-chain amyloidosis presenting with rapidly progressive submucosal hemorrhage of the stomach. Asian J Surg. 2016;39:113–5.CrossRefPubMedGoogle Scholar
  15. 15.
    Maleszewski JJ. Cardiac amyloidosis: pathology, nomenclature, and typing. Cardiovasc Pathol. 2015;24:343–50.CrossRefPubMedGoogle Scholar
  16. 16.
    Yildiz R, Yildirim B, Karincaoglu M, et al. Brain natriuretic peptide and severity of disease in non-alcoholic cirrhotic patients. J Gastroenterol Hepatol. 2005;20:1115–20.CrossRefPubMedGoogle Scholar
  17. 17.
    Barbosa M, Guardado J, Marinho C, et al. Cirrhotic cardiomyopathy: isn’t stress evaluation always required for the diagnosis? World J Hepatol. 2016;8:200–6.CrossRefPubMedPubMedCentralGoogle Scholar

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

Personalised recommendations