Rusznák M, Jakó J, Francz M, Babicz T
Nyíregyháza III. sz. Belgyógyászati Osztály/Kardiológia. Szabolcs-Szatmár-Bereg megyei Onkormányzat Jósa András Kórház Rendelöintézet.
Orv Hetil. 1993 May 9;134(19):1033-6.
A case of a 40-year-old female is reported. Her symptoms started with abdominal complaints, loss of weight, general weakness, then gradually polyneuropathy, worsening hypotonia and systolic heart murmur developed. The bone marrow showed 20-25% infiltration by myeloma. An IgG lambda type paraproteinaemia was diagnosed using immunelectrophoresis. The ejection systolic murmur was caused by restrictive cardiomyopathy--characteristic of primary amyloidosis--with obstruction of the outflow tract of the left ventricle. Echocardiographic changes took 11 months to develop. The patient's status became dominated by severe hypotonia and she died as a result of heart failure. The post mortem examination confirmed amyloidosis with myeloma. Histopathological examination of the myocardium, coronary and other arteries revealed severe changes with AL-type amyloid deposits. The case demonstrates that amyloidosis can lead not only to restrictive cardiomyopathy but obstruction of the outflow tract of the left ventricle.
