A 69-year-old Caucasian man was admitted to our Unit from the Emergency Department. He had been suffering from fever, dyspnea, fatigue, and dizziness for 10 days. He had no relevant medical history until the previous month, when he developed intermittent fever with chills. Ceftriaxone was administered at home without benefit. A few days before hospitalization, the patient's clinical condition worsened. On admission, the patient was confused, jaundiced, and had lower-limb edema.
Physical examination revealed jugular turgor, thready pulse with tachycardia, and hypotension (heart rate, 110 beats/minute; blood pressure, 90/60 mmHg) (Beck's triad). Chest auscultation revealed tachypnea (respiratory rate, 28 breaths/minute) and bilateral basal crepitations. Abdominal palpation disclosed hepatomegaly, splenomegaly, and a dull percussion sound in the lower abdominal quadrants.
Routine blood tests showed elevated white blood cells (2146 x 10^3/muL; neutrophils, 91.1%), normal Hb and PLTs, hyperglycemia, hyperbilirubinemia (bilirubin 5.50 mg/dl; 53% direct), and signs of hepatic dysfunction (aspartate aminotransferase (AST) 93 units/L, alanine aminotransferase (ALT) 119 units/L, gamma glutamyl transferase-GGT 285 units/L, serum albumin 2.9 g/dl, and INR 1.81). NT-pro-BNP was increased (2901 pg/ml), whereas cardiac-specific troponin was in the normal range (Table 1).
Since the electrocardiogram (ECG) identified high frequency sites during atrial fibrillation, digoxin and low-molecular-weight heparin (LMWH) were administered. Abdominal ultrasonography (US) revealed signs of hepatic disease (parenchymal inhomogeneity and increased diameters), splenomegaly (longitudinal diameter = 20 cm), and ascites. Heart US showed a difficult contraction and reduced diameter of the right ventricle with right atrium diastolic collapse and no inspiratory changes in the vena cava diameter. Chest CT scan demonstrated a retrosternal mediastinal mass containing calcifications and air bubbles. This mass did not show a cleavage plane over the heart but compressed it and shifted it to the left. Bilateral pleural effusion was also observed (Figure 1). Within a few hours, the blood pressure progressively decreased to 70/40 mmHg; dopamine (7 microg/kg/min) and oxygen (12 L/min) were given, and ECG monitoring was instituted. Urgent pericardiotomy was performed. Constrictive purulent-like pericardium and a firm mass with calcifications were removed. The patient rapidly improved: alertness, arterial pressure, and pulse rate normalized but he still had edema. NT-pro-BNP decreased in a couple of weeks (1430 pg/dl).
Because of the fever, antibiotic treatment was started, and insulin was administered for persistent hyperglycemia. A good response to the therapy was observed and paralleled a reduction of the WBC (11 x 103/microl) and an improvement of liver function, as indicated by a decrease of AST, ALT, and GGT. However, the Hb value (8 g/dl) and PLT count (80 x 103/microl) also decreased, with 3.1% of reticulocytes. Simultaneously, the albumin value was reduced (1.8 g/dl), whereas total bilirubin increased (9.1 mg/dl, 67% indirect), with a positive Coombs test for direct IgG. Therefore, albumin was administered.
One of the possible causes of effusive-constrictive pericarditis is tuberculosis (TBC). Since negative QuantiFERON ruled out classic TBC, we hypothesized atypical pericardial TBC or a fungal pericarditis. However, all viral (included HIV), microbiological, and cultural tests performed failed to reveal any infections.
Gamma-proteins were increased (30.8% vs 10-20% normal values) on serum protein electrophoresis and contained a monoclonal component (M component) accounting for 23.3% (1.64 g/dl) (Figure 2). Serum immunofixation demonstrated that the M component was IgG-lambda. Mild renal failure was present with proteins in the urine (1.56 g/24 h) that contained the Bence Jones as free lambda chains. Our analysis showed that the patient had had previous hepatitis A and B infections but not hepatitis C. No bacteria were demonstrated at blood cultures.
The culture test of the pericardial effusion demonstrated the Salmonella species (Salmonella suinis), so imipenem and ciprofloxacin were given. A sample of periumbilical fat was taken and histologically analyzed: amyloid AL deposits were found as positive Congo red and apple-green birefringent areas under polarized light (Figure 3).
Bone marrow (BM) biopsy showed 4% plasma cells (CD138 and lambda light chain positive) but not amyloid deposits. Skeleton radiological analysis was negative for bone lesions.
PET-CT with 18 fluorodeoxyglucose (FDG) showed a low uptake only in the mediastinum behind the sternum close to the heart. Optical microscope histological examination of the mediastinal mass showed hematoxylin-eosin pink amorphous material which was Congo-red positive and apple-green birefringent and included some granulocytes and few plasma cells (CD138 and lambda light chain positive) near the vessels.
We decided to start treatment according to the chemotherapy scheme VEL/DEX (bortezomib 1.3 mg/m2 i.v. on days 1, 4, 8, and 11; dexamethasone 40 mg i.v. on days 1, 2, 4, 5, 8, 9, 11, and 12).
The patient demonstrated good compliance and had a good response. The M component progressively lowered (12.2%, 0.67 g/dl), renal function improved, and urine proteins reduced. Epoetin A treatment was started to prevent a further decrease of Hb values.
At discharge, Hb, PLT, and serum bilirubin were stable; AST and ALT levels were normal; the pleural effusion, dyspnea, tachypnea, and edema had disappeared.
The patient underwent 4 cycles of Vel/Dex yielding a good clinical response. He slowly went back to work, with a good performance status.