000			01654cam a2200217 4500
001			NMDX5855
008			120401t2011 xxu||||| |||| 00| 0 eng d
022			_a17508460
100			_aLee, L.
240			_aBritish Journal of Hospital Medicine
245			_aCoexistent asymptomatic myeloma and hereditary cardiac amyloidosis: an unusual case of heart failure.
260			_c2011
500			_aNMUH Staff Publications
500			_a72
520			_aA 76-year-old Afro-Caribbean man presenting with heart failure was diagnosed with isolated cardiac amyloid. He had evidence of myeloma on bone marrow biopsy suggesting AL amyloid, the commonest type of systemic amyloidosis, as the underlying cause. He had no other myeloma-related organ damage. However, endocardial biopsy revealed amyloid fibrils composed of transthyretin and genetic typing established heterozygozity for the valine to isoleucine mutation at position 122 (Val122Ile). The diagnosis was therefore hereditary systemic amyloidosis as a result of a genetic transthyretin variant (ATTR) causing cardiac amyloidosis and coexistent asymptomatic myeloma. This requires symptomatic treatment of heart failure only. This article discusses a rare cause of heart failure and uses this case to illustrate that histological confirmation of the amyloid-causing protein is essential. Mistaken assumption of AL amyloid could have resulted in inappropriate cytotoxic therapy targeting the plasma cell clone.
700			_aAziz, M.
700			_aWechalekar, A.
700			_aRabin, N.
856			_uhttps://www.ncbi.nlm.nih.gov/pubmed/22083004
856			_uhttp://ferriman.wufoo.com/forms/r7x3a7/
999			_c75372 _d75372