20 days ago, the patient felt chest tightness, coughing, and sputum coughing discomfort after being cold. His symptoms did not be released significantly after resting. Then, He went to the Redcross Hospital of Qinghai Province and was diagnosed with "chronic bronchitis, emphysema, arrhythmia, splenomegaly". Half month later, the symptoms appeared, the patient had chest tightness, cough, and sputum symptoms worse than before, no chest pain and hemoptysis, no abdominal distension and abdominal pain. For further treatment, he went to the Qinghai University Affiliated Hospital.
Physical examination showed temperature 38°C, pulse rate 86 bpm, breathing rate 30 bpm and blood pressure 140/90mmHg. Consciousness, poor mental state, cyanotic lips, barrel chest, symmetrical voice tremor on palpation and blister sound and wheezing in both lungs. The heart rate was 86 bpm on auscultation, the rhythm is regular, and there were no abnormal murmurs in each valve area. In addition, the jugular vein was distended and the hepatic jugular vein reversing sign was positive. Abdominal tension and rebound pain were negative, the liver was untouchable under ribs, but the spleen under ribs was touched (line I measured 36.7mm, line II measured 78mm, and line III measured 21mm). Moreover, both lower limbs were edema, and the lower left limb was more obvious.
White blood cells counts 21.37×109/L, neutrophil counts 18.52×109/L, red blood cell counts 6.90×1012/L, hemoglobin 173g/L, hematocrit 51.30%, platelet counts 704×109 /L, lactate dehydrogenase 695U/L, uric acid 506umol/L, D-dimer 19.5mg/L, fibrinogen content 4.100g/L, C-reactive protein 67.70mg/L, procalcitonin 0.45 ng/mL, TnI 0.240ng/mL, N-terminal pro-brain natriuretic peptide 10900 ng/L. Echocardiography: The left and right atriums were enlarged, the tricuspid valve has a small amount of regurgitation, and the pulmonary valve has a small amount of regurgitation. There were no obvious abnormalities in the deep veins of the lower extremities. Pulmonary artery Computed Tomography Angiography (CTA) showed segmental visualization of the remote pulmonary artery of posterior right upper lobe. Bilateral pleural effusion, more obvious on the left, pulmonary hypertension, and the diameter of the main pulmonary artery is about 38.0 mm (Fig. 1). A plain CT scan of the abdomen showed fluid in the abdominal cavity; splenomegaly, multiple low-density changes in the spleen; portal hypertension (Fig. 3). The patient refused to undergo further enhanced CT examination of the abdomen. Therefore, he was given low-molecular-weight heparin 6000AxaIU (once every 12 hours), cefoperazone sodium and sulbactam sodium 3.0g intravenously (once every 8 hours), blood vessel expansion, diuresis and nasal cannula oxygen therapy, and the symptoms was released.
The bone marrow aspiration and biopsy were performed. The results of bone biopsy showed the possibility of Myeloproliferative Neoplasms (MPN). Then, he was recommended to complete the relevant examination to exclude MPN. BCR-ABL fusion gene qualitative test was negative. Bone marrow tissue pathology diagnosis report showed that hematopoietic tissue was obviously active, granulocytes, erythroid and megakaryocytes were all proliferating, and macronuclei and multi-megakaryocytes were seen, and the gene test showed JAK2 V617F positive. He was treated with 100 mg aspirin per day and 0.25 g hydroxyurea per day. After he discharged from the hospital, rivaroxaban was added to 10 mg oral anticoagulant therapy per day.
One month later, his chest tightness, cough, and sputum expectoration released significantly, re-examination of the pulmonary artery CTA showed the disappear of segmental visualization of the remote pulmonary artery of posterior right upper lobe, and there were no filling defects in the two pulmonary arteries. At the same time, the pulmonary hypertension reduced compared with the front, with the diameter of the main pulmonary artery about 32.5 mm, and the left pleural effusion was significantly reduced (Fig. 2). White blood cells 10.60×109/L, neutrophil count 8.45×109/L, neutrophil percentage 79.7%, red blood cell count 5.24×1012/L, hemoglobin 157g/L, hematocrit 49.2%, platelet count 233×109/L, he still refused to complete the enhanced abdominal CT examination. The rivaroxaban, hydroxyurea and allopurinol tablets were given outside the hospital.