Advances in Clinical and Experimental Medicine

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Advances in Clinical and Experimental Medicine

2014, vol. 23, nr 2, March-April, p. 235–244

Publication type: original article

Language: English

The Occurrence of AL Amyloidosis (Light-Chain Amyloidosis) in Patients with Multiple Myeloma in Lower Silesia Region, Poland

Lidia Usnarska-zubkiewicz1,A,C,D, Jadwiga Hołojda2,B,C, Michał Jeleń3,B, Anna Zubkiewicz-zarębska4,B,G, Jakub Dębski1,B, Kazimierz Kuliczkowski1,A,F

1 Department of Hematology, blood Neoplasms and bone Marrow Transplantation, Wroclaw Medical University, Poland

2 Hematology Department of district Specialist Hospital, Legnica, Poland

3 Division of Pathomorphology and Oncological cytology, Wroclaw Medical University, Poland

4 Department of Infectious diseases, Hepatology and acquired Immune deficiencies, Wroclaw Medical University, Poland


Background. The incidence of amyloidosis is difficult to determine because the disease is often undiagnosed or diagnosed incorrectly. In Polish studies, there are no statistics and analyses of the factors that may influence the development of amyloidosis in patients with multiple myeloma
Objectives. The goal of this study was to estimate the incidence of aL amyloidosis in MM patients in Lower Silesia region.
Material and Methods. 70 patients treated at the department of Hematology, Provincial Hospital in Legnica and the department of Hematology, blood Neoplasm and bone Marrow Transplantation, Medical University in Wroclaw were enrolled in the survey. 37 patients were newly diagnosed, 33 had been treated for 2–34 months. The basis for the diagnosis of amyloidosis was the presence of green colored amyloid deposits in the polarized light microscope in the adipose tissue (received from abdominal fold and stained with congo red).
Results. amyloidosis was diagnosed in 18 (25.7%) patients with MM, 9/9 f/M, aged 47–83 years. 6 (33%) pts with amyloidosis had newly diagnosed MM, in 12 (67%) progression of the disease was diagnosed. amyloidosis occurred significantly more often (p = 0.048) in already treated patients. The odds ratio (OR) was 2.95. amyloidosis occurred most frequently in patients with Igg myeloma (67%), (OR = 1.98), was more often found in patients with kappa light chain versus lambda, respectively 67% and 33%. The probability of amyloidosis in patients with clinical stage III was 1.5 times higher (p = 0.05) than in other stages (OR = 1.5), in persons with renal dysfunction was twice as high (OR = 2.4) compared to the renal competence group (p = 0.05).
Conclusion. aL amyloidosis in the course of MM occurs in Lower Silesia region with a comparable rate to other regions of the world. It is significantly more often diagnosed in patients with relapsed or refractory disease, in persons with clinical stage III and with renal failure.

Key words

amyloidosis, multiple myeloma, epidemiology, Lower Silesia, Poland.

References (26)

  1. Merlini G, Belloti V: Molecular mechanismus of amyloidosis N Engl J Med 2003, 349, 583–596.
  2. Kyle RA, Gertz MA: Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol 1995, 32, 45–59.
  3. Pepys, MB: Pathogenesis, diagnosis and treatment of systemic amyloidosis. Philos Trans R Soc Lond b biol Sci 2001, 356, 203–210.
  4. Abraham RS, Katzmann JA, Clark RJ, Bradwell AR, Kyle RA, Gertz MA: Quantitative analysis of serum free light chains. a new marker for the diagnostic evaluation of primary systemic amyloidosis. am J clin Pathol 2003, 119, 274–278.
  5. Kyle RA, Linos A, Beard CM: Incidence and natural history of primary systemic amyloidosis in Olmstead county, Minnesota, 1950 through 1989. blood 1992, 79, 1817–1822.
  6. Gertz MA, Lacy MQ, Dispenzieri A: amyloidosis: diagnosis and management. clin Lymphoma Myeloma 2005, 6, 208–219.
  7. Mayo MM, Johns GS: Serum free light chains in the diagnosis and monitoring of patients with plasma cell dyscrasias. contrib Nephrol. basel, Karger 2007, 153, 44–65.
  8. Gertz MA, Merlini G, Treon SP: amyloidosis and Waldenström’s macroglobulinemia. Hematology. am Soc Hematol Educ Program 2004, 257–282.
  9. Kurusu A, Hamada T, Yamaji K: a case of primary immunoglobulin light chain amyloidosis with a delayed appearance of bence Jones protein in urine. Nephrology 2004, 9, 122–125.
  10. Barosi G, Boccarodo M, Cavo M: Management of multiple myeloma and related-disorders: guidelines from the Italian Society of Hematology (SIE), Italian Society of experimental Hematology (SIES) and Italian group for bone Marrow Transplantation (gITMO). Haematologica 2004, 89, 717–741.
  11. Iwahashi N, Tome E, Nagasaka T: Massive hemorrhagie and pseudo-obstruction of the small intestine caused by primary aL amyloidosis associated with gastric cancer. Surg Todayic report and cause 2004, 34, 871–874.
  12. Duston MA, Skinner M, Shraham T, Cohen AS: diagnosis of amyloidosis by abdominal fat aspiration: analysis of 4 years experience. am J Med 1987, 82, 412–441.
  13. Merlini G, Stone MJ: dangerous small-b cell clones. blood 2006, 349, 583–596.
  14. Müller AMS, Geibel A, Neumann HPH: Primary (aL) amyloidosis in Plasma cell disorders. The Oncologist 2006, 11, 824–830.
  15. Puchtler H, Sweat F: congo red as a stain for fluorescence microscopy of amyloid. cytochem 1965, 13, 693–684.
  16. Rajkumar SV, Gertz MA, Kyle RA: Primary systemic amyloidosis with delayed progression to multiple myeloma. cancer 1998, 82, 1501–1505.
  17. Vela-Ojeda J, Garcia-Ruiz MA, Padilla-Gonzales V: Multiple myeloma associated amyloidosis is an independent high-risk prognostic factor. ann Hematol 2009, 88, 59–66.
  18. Gertz MA, Greipp GR: Hematological malignancies: multiple myeloma and related plasma cell disorders. Springer Verlag 2004.
  19. Desikan KR, Dhodapkar MV, Hough A: Incidence and impact of light chain associated (aL) amyloidosis on the prognosis of patients with multiple myeloma treated with autologous transplantation. Leuk Lymp 1997, 27, 315–319.
  20. Madan S, Dispenzieri A, Lacy MQ: clinical features and treatment response of light chain (aL) amyloidosis diagnosed in patients with previous diagnosis of multiple myeloma. Mayo clin Proc 2010, 85, 232–328.
  21. Dubrey SW, Cha K, Anderson J: The clinical features of immunoglobulin light chain (aL) amyloidosis with heart involvement. QJM 1998, 91, 141–157.
  22. Kracker D, Litbarg N, Picken MM: amyloidosis in ankylosing spondylitis: unexpected findings underscoring the importance of typing amyloid deposits. amyloid 2006, 13 Suppl 1, 38a.
  23. Quinton R, Siersema PD, Michiels JJ, Ten Kate FJWW: Renal aa amyloidosis in a patient with bence Jones proteinuria and ankylosing spondylitis. J clin Pathol 1992, 45, 934–946.
  24. Satoskar AA, Burdge K, Cowden DJ, Nadasdy GM, Hebert LA, Nadasdy T: Typing of amyloidosis in renal biopsies: diagnostic pitfalls. arch Pathol Lab Med 2007, 1319, 17–22.
  25. Abraham RS, Geyer SM, Price-Troska TL, Allmer C, Kyle R, Gertz MA: Immunoglobulin light chain variable(V) region genes influence clinical presentation and outcome in light chain – associated amyloidosis (aL). blood 2003, 101, 3801–3808.
  26. Bahlis NJ, Lazarus HM: Multiple myeloma associated aL amyloidosis: is distinctive therapeutic approach warranted? bone Marrow Transpl 2006, 38, 7–15.