A 69-year-old female was admitted to our hospital for chest distress accompanied by limb swelling for 2 years and exacerbation for 2 months. She had no history of hypertension, diabetes, chronic obstructive pulmonary disease and had no history of smoking and drinking familial hereditary diseases.
Physical examination: BP 80/50mmHg, jugular veins were not distended, the lower lungs were percussive dullness with some damp rales. The heart rate was 82 beats per minute, the rhythm was absolutely irregular and the strength of the first heart sound varied. The abdomen was soft, with positive mobile dullness, and symmetrical edema of both lower extremities were documented.
Laboratory examinations: Blood cell analysis was normal. Biochemical: potassium 3.76mmol/L, sodium122.7mmol/L, albumin 25.8g/L, creatinine161umol/L, uric acid 592umol/L. TnI 0.054ng/mL (0.010--0.023). NT-ProBNP 30700 pg/mL. Thyroid function: FT3 3.02pmol/L, FT4 22.88pmol/L, TSH 6.30uIU/Ml. Urine-free light chain KAPPA 89.2mg/L, LAMBDA15.3mg/L, serum-free light-chain KAPPA 27.2mg/L, LAMBDA 315.0mg/L. Immunofixation electrophoresis: detection of monoclonal immunoglobulin antibodies. Immunoglobulin was normal. Anti-dsDNA antibodies(±).
Abdominal fat biopsy: Congo red staining(-). Bone marrow biopsy: granulocytes proliferate actively, megakaryocytes decrease, a small number of abnormal plasma cells, abnormal CD56, and Ig light chain LAMBDA expression were up-regulated.
Imaging examinations: electrocardiogram(ECG) showed atrial fibrillation, low voltage of limb lead(Figure 1). Echocardiography showed double atrium and right ventricular enlargement, aortic valve calcification with mild regurgitation, left ventricular wall thickening, left ventricular thrombosis, left ventricular EF 36%, moderate to severe tricuspid valve, mild pulmonary valve, mild-to-moderate mitral regurgitation, moderate pulmonary hypertension, small pericardial effusion(Figure 2). Coronary angiography showed 50% stenosis of the middle left anterior descending artery. Cardiac magnetic resonance(MR) showed double atrial enlargement, left ventricular EF was 23%, diffuse asymmetric left ventricular myocardial thickening, diffuse delayed enhancement of the entire myocardium, myocardial T1 prolonged, myocardial extracellular volume fraction was significantly increased. CT showed a large amount of pleural and pelvic effusion(Figure 3,4).
Treatment
After admission, the patient suffered from abdominal distension and anorexia, and resistant hypotension, oliguria, and hyponatremia. Vasoactive drugs were given to maintain blood pressure, supplemented with concentrated sodium, infused with albumin, and thoracic and abdominal cavity puncture and drainage. The patient could not receive chemotherapy due to poor general condition. The patient died two months later.