Systemic amyloidosis due to unknown multiple myeloma in small bowel pseudo-obstruction: case report

Giuseppe Caparrotti, Diego Di Maria, Elia Iovane, Clemente Martone, Francesco Graziani, Giuseppina De Falco, Daniela Esposito, Claudio Marotta, Giuseppina Papa, Isidoro Orabona, Davide Albino Rossetti, Nicola Armiento, Gennaro Ferrara, Pasquale Pezzella

DOI: https://doi.org/10.7175/cmi.v10i1.1213

Abstract

Amyloidosis is a pathologic diagnosis characterized by extracellular deposition of insoluble protein fibrils in various organs and tissues. There are two main forms of amyloidosis, primary amyloidosis, and secondary amyloidosis. Gastrointestinal involvement is common in both amyloidosis forms.

We describe the case of a 78-year-old woman taken to the operating room for small bowel obstruction, found to have pseudo-obstruction and enteritis. Exploratory laparotomy revealed gastric mass and histological examen showed extensive amyloid deposition consistent with amyloidosis. Hematological evaluation revealed unknown multiple myeloma. This case report and literature data suggest to perform a hematological examination in patients with amyloidosis diagnosis to exclude a multiple myeloma or other plasma cell disorders

Keywords

Myeloma; Amyloidosis; Pseudo-obstruction; Hypogammaglobulinemia; Congo red stain

Full Text:

HTML PDF

References

  • Glenner GG. Amyloid deposits and amyloidosis: the beta-fibrilloses. N Engl J Med 1980; 302: 1333-43; http://dx.doi.org/10.1056/NEJM198006123022403
  • Eastwood H, Cole KR. Staining of amyloid by buffered Congo red in 50 percent ethanol. Stain Technol 1971; 46: 208-9; http://ncbi.nlm.nih.gov/pubmed/4105512
  • Falk RH, Conenzo RL, Skinner M. The systemic amyloidosis. N Engl J Med 1997; 337: 898-909; http://dx.doi.org/10.1056/NEJM199709253371306
  • Ogawa S, Murakami T, Inoshima Y, et al. Effect of heating on the stability of amyloid A (AA) fibrils and the intra- and cross-species transmission of AA amyloidosis. Amyloid 2015; 22: 236-43; http://dx.doi.org/10.3109/13506129.2015.1095735
  • Merlini G, Seldin DC, Gertz MA. Amyloidosis: pathogenesis and new therapeutic options. J Clin Oncol 2011; 29: 1924-33; http://dx.doi.org/10.1200/JCO.2010.32.2271
  • Ebert EC, Nagar M. Gastrointestinal manifestations of amyloidosis. Am J Gastroenterol 2008; 103: 776-87; http://dx.doi.org/10.1111/j.1572-0241.2007.01669.x
  • Lin X, Mao Y, Qi Q, et al. Primary systemic amyloidosis initially presenting with digestive symptoms: a case report and review of the literature. Diagn Pathol 2015; 10: 174; http://dx.doi.org/10.1186/s13000-015-0407-9
  • Van Gameren II, Hazenberg BP, Bijzet J, et al. Diagnostic accuracy of subcutaneous abdominal fat tissue aspiration for detecting systemic amyloidosis and its utility in clinical practice. Arthritis Rheum 2006; 54: 2015-21; http://dx.doi.org/10.1002/art.21902
  • Bhole MV, Sadler R, Ramasamy K. Serum-free light-chain assay: clinical utility and limitations. Ann Clin Biochem 2014;51:528-42. http://dx.doi.org/10.1177/0004563213518758
  • Koppelman RN, Stollman NH, Baigorri F, et al. Acute small bowel pseudo-obstruction due to AL amyloidosis: a case report and literature review. Am J Gastroenterol 2000; 95: 294-6; http://dx.doi.org/10.1111/j.1572-0241.2000.01712.x
  • Cowan AJ, Skinner M, Seldin DC, et al. Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience. Haematologica 2013; 98: 141-46; http://dx.doi.org/10.3324/haematol.2012.068155
  • Rajkumar SV, Dispenzieri A, Kyle RA. Monoclonal gammopathy of undetermined significance, Waldentrom macroglobulinemia, AL amyloidosis, and related plasma cell disorders: diagnosis and treatment. Mayo Clin Proc 2006; 81:693-703; http://dx.doi.org/10.4065/81.5.693
  • Rajkumar SV, Gertz MA, Kyle RA. Primary systemic amyloidosis with delayed progression to multiple myeloma. Cancer 1998; 82: 1501-5; http://dx.doi.org/10.1002/(SICI)1097-0142(19980415)82:8<1501::AID-CNCR11>3.0.CO;2-8

Refbacks

  • There are currently no refbacks.




© SEEd srl