A 41-year-old male came to the hospital for fatigue, satiety and abdominal distension in March 2018. Palpation revealed that the lower edge of his spleen reached the umbilicus level. His chest computed tomography (CT) scan showed no abnormality. Bone marrow biopsy showed remarkably increased age-adjusted cellularity with granulocyte proliferation, decreased erythropoiesis, a large number of megakaryocytes atypia, grade 0 fibrosis, and the JAK2V617F mutation. He was diagnosed with Pre-PMF. His Dynamic International Prognostic Scoring System (DIPSS) risk group was intermediate-1. He was started on rux (20 mg, twice daily).
Fever and productive cough started in early July and lasted approximately one week. He was hospitalized on 12th July for antibiotics. Laboratory tests showed white blood cells (WBC) 16.11x109/L, erythrocyte sedimentation rate (ESR) 40 mm/h, hypersensitive C-reactive protein 107.7 mg/L, and procalcitonin (PCT) 0.76 microg/L. Blood lymphocyte subsets showed an absolute total T cell count of 347/muL (normal range: 995-2860/muL) and CD4+ T cell count of 208/muL (normal range: 550-1440/muL). Sputum acid-fast bacilli (AFB) smears were negative, but interferon gamma release assay (IGRA, T-SPOT.TB) was positive. Transient syncope unexpectedly occurred the next morning. Multiple deep venous thrombosis was later confirmed by ultrasound in his left lower extremity. Enhanced CT showed pulmonary thromboembolism in his right upper and inferior pulmonary artery, bilateral pleural effusion, pulmonary infection, multiple enlarged mediastinal lymph nodes and soft tissue shadows that surrounded his left main bronchus (Figure 1A and C). CT also revealed frontal lobe hemorrhage. He received low molecular weight heparin instantly, and rux was discontinued from then on. To prevent the occurrence of lethal complications, he was transferred to vascular surgery for inferior vena cava filter implantation. A bronchoesophageal fistula on his left main bronchus was later discovered by iodine contrast esophagogram in response to persistent dry cough, and then he received nasal jejunal nutrition (Figure 1B).
Hydroxyurea was introduced to control the WBC count in September. Given that infection work-up was negative, fiberoptic bronchoscopy was chosen to determine whether he was infected with TB. However, bronchoalveolar lavage fluid (BALF) specimens tested negative for culture, AFB and GeneXpert assay. A tracheobronchial lymph node biopsy finally confirmed Mycobacterium tuberculosis (MTB) by polymerase chain reaction. He was diagnosed with tracheobronchial tuberculosis (TBTB) and received a 12-month antituberculosis therapy (ATT) involving isoniazid, rifampicin, ethambutol and levofloxacin. Another two bronchoalveolar lavages were performed during ATT. BALF was negative for all relevant results, including mycobacterial ribonucleic acid (RNA) and deoxyribonucleic acid (DNA). The mediastinal lymph nodes' size also decreased (Figure 2). In July 2019, rux (10 mg, twice daily) was reintroduced due to splenomegaly and abdominal distension. His DIPSS was intermediate-1 at that time.
Imaging appearances in case 1. (A) Enhanced chest computed tomography (CT) showing the pulmonary thromboembolism (arrow) in right inferior pulmonary artery and bilateral pleural effusion.