A 19-year-old girl (162 cm, 40 kg) came to our hospital due to suffering from fever, intermittent headache and unclear vision for some days. She had a two-month history of hypertension with the highest blood pressure of 200/110 mmHg. The biochemical parameters were white blood cell count 14.56 × 109/l,platelet(PLT) count 1112 × 109/l, hemoglobin 81 g/l, C-reaction protein(CRP) 116.3 mg/l, urine catechol amine 2947.20 nmol (reference range: 94.5-238.3 nmol), urine noradrenalin 2916.82 nmol (reference range: 80.3–164 nmol), urine adrenalin 30.38 nmol (reference range: 12.5–70.4 nmol), urine vanillin mandelic acid 212.95 µmol (reference range < 68.6 µmol) at 24 h. The abdominal enhanced computed tomography(CT) showed that an enhanced mass was found at the right anterior lower adrenal gland, based on which the diagnosis of paraganglioma was suggested for this patient (Fig. 1). A question came to our mind:was this girl's fever and thrombocytosis due to paraganglioma or hematologic diseases? In order to make clear whether the patient was complicated with hematologic disease, We made a bone marrow puncture, which indicated normal bone marrow .Thus, It made sure that the patient’s clinical manifestation was all caused by paraganglioma.
The major difference between this patient and the other paraganglioma patient is the long-term intermittent fever and severe thrombocytosis. These characteristics of paraganglioma were rarely reported previously. This patient needs surgical treatment, and we did preoperative preparation and treatment for the young girl. To abatement the fever,antibiotic,non-steroidal anti-inflammatory drugs(NSAIDs) and dexamethasone were provided for the patient, however, These were not effective. She also orally took hydroxycarbamide and apheresis platelets to decrease platelets and prevent thrombosis, and α-adrenergic receptor blocker with calcium channel blocker to decrease blood pressure. 7 days later, the patient had the resection of right side paraganglioma with abdomen under general anesthesia. It took 40 minutes from tumor isolation to tumor vessel ligation. During this period the highest blood pressure was 185 / 118 mmHg, the heart rate was 120 times / min, and phentolamine, nitroglycerin, and magnesium sulfate were added up to 120 mg, 0.8 mg and 2.5 g, respectively. After tumor resection, the blood pressure decreased, and norepinephrine was pumped at 0.15–0.25 µg/(kg·min) to maintain blood pressure. A 5 × 4 cm tumor was resected finally.The operation was successful.After the operation,the little girl had no fever and the platelet recover to nomal level. Changes in perioperative related laboratory parameters were showed in table I.So we finally confirmed that her clinical manifestations were only caused by paraganglioma. The patient was discharged at postoperative day 11.
Table I Changes in perioperative related laboratory parameters
| Before operation | After operation | Discharge | Reference range |
PLT(*109 /l) | 1055 | 418 | 247 | 125–350 |
FIB(g/l) | 9.52 | 5.99 | 4.09 | 2–4 |
MA(mm) | 87.6 | 81 | 69.9 | 50–70 |
CI | 3.6 | 3.7 | 1.4 | -3-3 |
hsCRP(mg/l) | > 10.4 | / | 2.43 | 0–3 |