A 66-year-old male patient who suffered recurrent pleural effusion for more than 6 months and coughed for 2 months was admitted to hospital for clear diagnosis and treatment. He was previously engaged in a job which exposed him to dust and talcum powder for a long time.
He underwent right thoracentesis and anti-infective treatment before admission. The patient's cough and shortness of breath were slightly relieved. He still experienced pleural effusion and had symptoms of cough and shortness of breath.
On physical examination, the patients' blood pressure was 110/80mm Hg and SaO2 was 96%. Respiratory rate was 18 times per minute. Heart rate was 87 times per minute. Temperature was 36.8 C. There was decreased breath sound at right lung base. There was no edema in lower limbs. Laboratory examinations revealed that complete blood count and serological data, including the C-reactive protein level and procalcitonin level, was within the normal range. Laboratory tests related to the immune system were also negative. The comprehensive metabolic panel, including total protein, liver function tests, showed pro-BNP of 1211pg/mL, D-dimer of 4.85ug/mL, total protein of 47.1 g/L, albumin of 31.9 g/L, globulin of 15.2 g/L, serum troponin I of 0.002ng/mL. Echocardiography indicated diastolic dysfunction. Chest X-ray taken at the time of admission demonstrated bilateral pleural effusion. There was much pleural effusion on the right side. During the hospitalization, the patient underwent thoracentesis 3 times on right side while 1 time on left side. The 1st thoracentesis which is performed on day 1 of admission drainaged egg-yellow effusion (Fig. 1). The biochemical examination showed lymphocyte count of 98%, monocyte count of 2%, glucose of 6.88 mmol/L, total protein 37.8 g/L, lactate dehydrogenase (LDH) of 96mmol/L, adenosine deaminase of 5.1U/L. The subsequent 2 thoracentesis also presented as egg-yellow effusion. The biochemical result were suggestive of exudative effusions for both of them. Cultures were also negative. Cytological examination of all pleural effusion samples revealed mesothelial cells and numerous lymphocytes without cancer cells.
For this patient with repeated pleural effusions, we considered the possibility of pneumonia effusion, or tuberculosis, or tumor?
Initial treatment was mainly based on anti-infective therapy in the early hospital stay. Levofloxacin was selected as antibiotics. However, we found that pleural effusion did not decrease but increased after a dynamic observation of pleural fluid using doppler ultrasound examination. As a result, we suspended the use of levofloxacin on day 14 of admission. Other diagnosis was suggested. In order to find the diagnosis, thoracoscope (day 5), GeneXpert Mycobacterium tuberculosis (MTB)/rifampicin (RIF) test on pleural effusion (day 13) and chest CT angiography (day 15) were all performed during early hospitalization. GeneXpert MTB/RIF test on pleural effusion was negative. Chest CT angiography demonstrated left lower pulmonary embolism (Fig. 2). The thorascopy showed hyperaemia and black tissue of the parietal pleura, that were biopsied. The specimen was submitted for cytopathologic valuation. Paraffin electron microscopy of pleural biopsy was consistent with pleural amyloidosis (Fig. 3). The lesion was mainly comprised of amyloid deposition. Congo red stain was positive in the pleural tissue (Fig. 4). When viewed under polarized light, the affected tissue typically shows apple-green birefringence after staining with Congo red (Fig. 5). The stainability of Congo red stain was not lost by potassium permanganate treatment, the lesion was pathologically confirmed to be amyloidosis, excluding the amyloid protein A (AA) type (Fig. 6). The patient refused bone marrow biopsy. We could not further distinguish whether the disease was primary or secondary, despite there were no significant clinical symptom of multiple myeloma. As a result, immunoglobulin light chain (AL) type amyloidosis of pleura was suggested.
The final diagnosis of this patient was AL pleural amyloidosis and left lower pulmonary embolism.
In aspect of pulmonary embolism, Nadroparin calcium combined Warfarin were performed as anticoagulative therapy. Thirteen days later, chest CT angiography did not see previous pulmonary embolism lesion (Fig. 7). And the patient's heart was functioning normally. In aspect of pleural amyloidosis, the patient was repeatedly advised to undergo bone marrow biopsy and pleurodesis. The patient refused and requested for discharge after serious consideration. The patient's shortness of breath was relieved at the time of discharge, and the pleural effusion was less than before. After discharge, the patient's phone could not be connected during our follow-up.