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Inhaled Epoprostenol as a Rescue Therapy for Acute Respiratory Distress Syndrome in a Patient with Acute Myeloid Leukemia, Leukostasis, and Tumor Lysis Syndrome
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A5272 - Inhaled Epoprostenol as a Rescue Therapy for Acute Respiratory Distress Syndrome in a Patient with Acute Myeloid Leukemia, Leukostasis, and Tumor Lysis Syndrome
Author Block: C. Tsai, B. Walsh; Pulmonary/Critical Care Medicine, Geisinger Medical Center, Danville, PA, United States.
Introduction: Respiratory events are frequent and may occur early in patients with newly diagnosed acute leukemia. Initial mortality rate for patients with acute myeloid leukemia (AML) and leukostasis in the first week ranges from 20-40% and increases to 80-90% in AML patients who develop acute respiratory failure requiring mechanical ventilation.
Case: A 64-year old female with past medical history significant for anxiety and hypothyroidism presented with dizziness, nausea, and fatigue. She was intubated for acute respiratory distress. Lab work revealed a white blood cell count of 147.67 K/uL with 20% myeloblasts, 17% metamyelocytes, and 5% myelocytes, hemoglobin 2.6 g/dL, platelets 84 K/uL, creatinine 1.9 mg/dL, and lactate dehydrogenase 1461 U/L. The patient was suspected to have AML, leukostasis, and tumor lysis syndrome (TLS). She was admitted to the intensive care unit (ICU) and leukopheresis was initiated. Arterial blood gas revealed pH 7.378, pCO2 35.6, pO2 of 48.2 on FiO2 100%. Transthoracic echocardiogram showed a mildly reduced ejection fraction of 40-44%. Chest imaging revealed diffuse bilateral opacities. Lung protective, low tidal volume ventilation was initiated for severe acute respiratory distress syndrome (ARDS). She had refractory hypoxemia despite multiple ventilator adjustments and neuromuscular blockade. Inhaled epoprostenol was initiated. Within two hours, FiO2 requirement was down to 80%. By the next morning, PaO2/ FiO2 was 190 on FiO2 of 50%. Neuromuscular blockade was discontinued. Within 24 hours of initiation, inhaled epoprostenol was titrated down and discontinued. Bone marrow biopsy confirmed AML and chemotherapy was initiated with cytarabine. Patient was extubated on hospital day 6 and transferred to the floor to continue chemotherapy.
Discussion: ARDS may develop early in patients with acute leukemia. Protective lung ventilation, prone positioning, and neuromuscular blockade have proven benefit in patients with ARDS. Extracorporeal membrane oxygenation remains a relative contraindication for patients with AML. Use of dexamethasone with chemotherapy has shown variable results. Selective pulmonary vasodilators (SPVs) are generally reserved for patients with refractory hypoxemia as their efficacy and use remains controversial. While there are no proven benefits of SPVs, improvement of PaO2/FiO2 after 24 hours of inhaled epoprostenol has been associated with lower mortality rate. To our knowledge, this is the first reported case of successful use of inhaled epoprostenol as rescue therapy for ARDS in a patient with AML, leukostasis, and TLS.
Conclusion: With high mortality rates in patients with AML and ARDS, inhaled epoprostenol should be considered in the presence of refractory hypoxemia.
Author Block: C. Tsai, B. Walsh; Pulmonary/Critical Care Medicine, Geisinger Medical Center, Danville, PA, United States.
Introduction: Respiratory events are frequent and may occur early in patients with newly diagnosed acute leukemia. Initial mortality rate for patients with acute myeloid leukemia (AML) and leukostasis in the first week ranges from 20-40% and increases to 80-90% in AML patients who develop acute respiratory failure requiring mechanical ventilation.
Case: A 64-year old female with past medical history significant for anxiety and hypothyroidism presented with dizziness, nausea, and fatigue. She was intubated for acute respiratory distress. Lab work revealed a white blood cell count of 147.67 K/uL with 20% myeloblasts, 17% metamyelocytes, and 5% myelocytes, hemoglobin 2.6 g/dL, platelets 84 K/uL, creatinine 1.9 mg/dL, and lactate dehydrogenase 1461 U/L. The patient was suspected to have AML, leukostasis, and tumor lysis syndrome (TLS). She was admitted to the intensive care unit (ICU) and leukopheresis was initiated. Arterial blood gas revealed pH 7.378, pCO2 35.6, pO2 of 48.2 on FiO2 100%. Transthoracic echocardiogram showed a mildly reduced ejection fraction of 40-44%. Chest imaging revealed diffuse bilateral opacities. Lung protective, low tidal volume ventilation was initiated for severe acute respiratory distress syndrome (ARDS). She had refractory hypoxemia despite multiple ventilator adjustments and neuromuscular blockade. Inhaled epoprostenol was initiated. Within two hours, FiO2 requirement was down to 80%. By the next morning, PaO2/ FiO2 was 190 on FiO2 of 50%. Neuromuscular blockade was discontinued. Within 24 hours of initiation, inhaled epoprostenol was titrated down and discontinued. Bone marrow biopsy confirmed AML and chemotherapy was initiated with cytarabine. Patient was extubated on hospital day 6 and transferred to the floor to continue chemotherapy.
Discussion: ARDS may develop early in patients with acute leukemia. Protective lung ventilation, prone positioning, and neuromuscular blockade have proven benefit in patients with ARDS. Extracorporeal membrane oxygenation remains a relative contraindication for patients with AML. Use of dexamethasone with chemotherapy has shown variable results. Selective pulmonary vasodilators (SPVs) are generally reserved for patients with refractory hypoxemia as their efficacy and use remains controversial. While there are no proven benefits of SPVs, improvement of PaO2/FiO2 after 24 hours of inhaled epoprostenol has been associated with lower mortality rate. To our knowledge, this is the first reported case of successful use of inhaled epoprostenol as rescue therapy for ARDS in a patient with AML, leukostasis, and TLS.
Conclusion: With high mortality rates in patients with AML and ARDS, inhaled epoprostenol should be considered in the presence of refractory hypoxemia.
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