A 55-year-old man with a history of exertional fatigue presented
with dyspnoea since 10 days ago. The electrocardiogram
showed low voltage on the limb leads I and aVL, and
QS-type waves on the precordial leads V1 to V3. The echocardiography
revealed the ejection fraction 59%, left ventricular
end-diastolic diameter 36 mm, and interventricular septum
15 mm.
The serum B-type natriuretic peptide was 1940 pg/
ml and the 24-hour urine protein was 3.69 g. Renal biopsy
showed amyloid deposition in glomeruli and mesangial cells
(panel A). Both the Congo red staining and potassium permanganate
staining were positive (panels B, C). The bone marrow
aspiration showed plasma blasts 0.4%, proplasmacytes 6.8%
and plasmacytes 17.6%. Flow cytometry showed that there
were 24.36% abnormal plasma cells which expressed kappa
light chain proteins (panels D, E, F). The patient was diagnosed
with systemic light chain amyloidosis with cardiac and renal
involvements and given melphalan and prednisone. His recovery
was uneventful.
Amyloidosis is a protein-folding disorder with more than organ
infiltrated by proteinaceous deposits, and the prognosis
of the disease is determined both by the organs involved and
the type of amyloid. Cardiac amyloid involvement carries the
worst prognosis of any involved organ. Early diagnosis is important
for improving the patient’s outcome.
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