Cancer Treatment Today

Amyloidosis can occur in the setting of multiple myeloma. AL amyloidosis may develop in patients with multiple myeloma (10%–15%) or may progress from monoclonal gammopathy of undetermined significance (MGUS) (9%).

Treating Cardiac Amyloidosis is fraught with complications.

The pathological biopsy is the only means to confirm myocardial amyloidosis. Myocardial amyloidosis can be confirmed by combining positive results on ECG, echocardiography, and MRI with biopsy tissue Congo Red staining, but negative results of Congo Red staining do not rule out myocardial amyloidosis. However, because of poor cardiac function in patients with myocardial amyloidosis, blind examinations such as that of kidney tissue, abdominal adipose tissue, and intestinal tissue are often used as substitutes for myocardial biopsy. The positivity rates of different tissue specimens stained with Congo Red were reported as follows: abdominal fat, 50–80%; bone marrow, 56%; rectum, 75%; kidney, 94%; carpal ligament, 82%; liver, 97%; small intestine, 83%; skin, 90%; sural nerve, 86%; and heart, 100%.
Some studies show no additional benefit from induction chemotherapy with respect to survival, hematological or organ response. To the contrary, and despite a delay in autograft of only 9 weeks with induction treatment, in one study, patients with cardiac amyloidosis appeared to suffer a survival disadvantage. In all, 13% of the patients treated with initial oral chemotherapy could not proceed to autograft because of disease progression ultimately leading to death. In a similar study, induction chemotherapy with VAD before autograft did not result in any increase in either hematological response or survival parameters. Based on these reports, it is recommended that transplant-eligible amyloidosis patients should proceed directly to stem collection with G-CSF alone without any prior induction chemotherapy.
For this reasohn, the diagnosis should be estblished before considering autologosu stemc ell transplantation instead of chemotehrapy/ meyloma therapy. A WHile a cardiac biopsy is not required, one of the other sites should be biopsied to confirm teh diagnosis.

Zhang Q, Qiao Y, Yan D, Deng Y, Zhang M, Xu P. Myocardial amyloidosis following multiple myeloma in a 38-year-old female patient: A case report. Open Med (Wars). 2020;15(1):396-402. Published 2020 May 16. doi:10.1515/med-2020-0125

Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol. 1995;32(1):45–59. [PubMed]Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol. 1995;32(1):45–59.

Gillmore JD, Wechalekar A, Bird J, Cavenagh J, Hawkins S, Kazmi M, et al. Guidelines on the diagnosis and investigation of AL amyloidosis. Br J Haematol. 2015;168(2):207–18.

Parrilla F, Calderon RE, Figueroa R, Gurrea C. Cardiac amyloidosis secondary to multiple myeloma. Bol Asoc Med P R. 2013;105(2):39-42. PMID: 23882988.

Bahlis, N., Lazarus, H. Multiple myeloma-associated AL amyloidosis: is a distinctive therapeutic approach warranted?. Bone Marrow Transplant 38, 7–15 (2006).