Longer Duration of Palliative Care in Patients With COPD Is Associated With Death Outside the Hospital.

Background: Patients with advanced chronic obstructive pulmonary disease (COPD) have a significant symptom burden despite maximal medical therapy, yet few are referred for concomitant palliative care. Objective: To evaluate the utilization and impact of palliative care on the location of death and to identify clinical variables associated with palliative care contact. Design: Retrospective chart review from 2010 to 2016 at the VA Western New York Healthcare System using ICD-9/10 diagnosis of COPD. Palliative care contact was identified by Z51.5 or stop code 353. Results: Only 0.5% to 2% of living patients received palliative care, increasing abruptly at death (6%). Lower diffusion capacity for carbon monoxide (DLCO) (greater emphysema) was associated with palliative care contact, independent of comorbid disease burden or age. Initial outpatient contact was associated with a longer duration of palliative care (P = .003) and death in a home-like setting. Outpatient palliative care was associated with more severe airflow obstruction (forced expiratory volume in 1 second, percent predicted [FEV1%]), whereas greater disease exacerbation frequency was associated with inpatient contact. COPD patients not referred to palliative care had a greater comorbid disease burden, similar FEV1%, fewer disease exacerbations, and a greater DLCO. Conclusion: Few patients with COPD received palliative care, similar to national trends. Initial outpatient palliative contact had the longest duration of care and death in the preferred home environment. The extent of emphysema (DLCO reduction) and more frequent disease exacerbations identified in patients were more likely to receive palliative care. Our study begins to define the benefits of palliative care in advanced COPD and confirms underutilization in the years before death, where a prolonged impact on the quality of life may be realized.

Case of haemophagocytic lymphohistiocytosis following Epstein-Barr virus infection.

Haemophagocytic lymphohistiocytosis (HLH) is a rare diagnosis that carries a high degree of mortality. We present this case of a previously healthy 22-year-old woman, who was admitted acutely ill to the hospital. One week prior, she had been seen by her primary care physician for fatigue and malaise. At that time, she was noted to have anterior and posterior cervical lymphadenopathy. She was referred to the emergency room and was diagnosed with acute Epstein-Barr virus (EBV) mononucleosis based on her clinical symptoms and positive heterophile antibody test. She was discharged after an uneventful 48-hour stay on the wards. She represented 7 days after discharge with cough, fatigue, nausea, vomiting, epigastric abdominal pain, diarrhoea, weight loss and subjective fevers. She had also reported haematemesis, epistaxis and melaena. Vital signs included temperature 36.9°C, blood pressure 90/50 mm Hg, heart rate 130 beats per minute and respiratory rate 32 breaths per minute. Physical examination was notable for an acutely ill appearing woman with scleral icterus, hepatosplenomegaly and palpable cervical and axillary lymphadenopathy. Complete blood count showed pancytopaenia with haemoglobin 59 g/L (normal 120-160 g/L), white blood cell count 2.7×109/L (normal 4-10.5×109/L) and platelet count 50×109/L (normal 150-450×109/L). The white blood cell count differential included 58% neutrophils (normal 38%-77%) with immature neutrophils in band form elevated at 45% (normal <14%), 16% lymphocytes (normal 20%-48%), 7% monocytes (normal <12%) and no eosinophils (normal <6%). Blood smear revealed anisocytosis, poikilocytosis and hypochromia. Coagulation panel showed elevated levels of d-dimer level at 1.39 µg/mL (normal <0.45 µg/mL), prolonged prothrombin time at 34.4 s (normal 11-15 s), prolonged activated partial thromboplastin time of 55.6 s (normal 25-34 s), prolonged international normalised ratio at 3.31 (normal <1.1) and low fibrinogen 60 mg/dL (normal >200 mg/dL). Lipid panel showed cholesterol at 114 mg/dL (normal 125-200 mg/dL), triglycerides 207 mg/dL (normal 30-150 mg/dL), high-density lipoprotein cholesterol 10 mg/dL (normal 40-60 mg/dL) and low-density lipoprotein cholesterol 63 mg/dL (normal <100 mg/dL). Other lab abnormalities included elevated ferritin of 6513 ng/mL (normal 10-150 ng/mL) and elevated lactate dehydrogenase of 1071 unit/L (normal 95-240 unit/L). Soluble interleukin-2 receptor alpha level was elevated at 60 727 units/mL (normal 223-710 units/mL). Fluorodeoxyglucose-positron emission tomography (FDG-PET) scan showed abnormal tracer localisation within the paratracheal, hilar, pelvic, abdominal and subcarinal lymph nodes, along with FDG-PET positive hepatosplenomegaly. A bone marrow biopsy showed hypercellular marrow (95% cellularity) with trilineage haematopoiesis, haemophagocytic cells, polytypic plasmacytosis and T-cell lymphocytosis, along with positive latent membrane protein-1 immunohistochemical staining for EBV. EBV quantitative DNA PCR showed >1 million copies. These findings were consistent with a diagnosis of HLH secondary to EBV infection. Despite intense therapy with the HLH-94 protocol, the patient expired from her illness after a prolonged hospital course.

Flip flop fungus sign: an FDG PET sign of benign pulmonary nodules.

A 71-year-old man was referred to pulmonary clinic for incidental findings of hypermetabolic lung nodule and mediastinal adenopathy on CT FDG PET performed for evaluation of cough. The patient underwent bronchoscopy with endobronchial ultrasound that was non-diagnostic. The patient was subsequently sent for video-assisted thoracoscopic lymph node biopsy notable for confluent caseating granulomas due to chronic infection by Histoplasma capsulatum Review of previous PDG PET was notable for the flip flop fungus sign-a PDG PET finding that could have altered the patients' clinical course by potentially avoiding the need for invasive surgical tissue diagnosis.

Pancreaticopleural fistula: an uncommon cause of amylase-rich pleural effusion.

Pancreaticopleural fistula (PPF) causing pleural effusion as a complication of chronic pancreatitis is a rare finding. We present this finding in a 52-year-old man with a medical history significant for alcohol abuse, acute on chronic pancreatitis and severe chronic obstructive pulmonary disease, who presented with worsening dyspnoea for 3 days. CT scan of the chest showed a new large right-sided pleural effusion. Thoracentesis was performed and pleural fluid analysis showed an amylase-rich, exudative pleural effusion. The effusion reaccumulated within 3 days necessitating repeat thoracentesis. Endoscopic retrograde chloangiopancreatography showed contrast leak through a single disruption in the dorsal pancreatic duct, suspicious for an underlying PPF. The patient underwent stenting of the pancreatic duct with subsequent resolution of right-sided pleural effusion.