Abstract Introduction The incidence of Acute Respiratory Distress Syndrome (ARDS) in patients with high-grade Myelodysplastic Syndrome (MDS) undergoing transformation to Acute Myeloid Leukemia (AML) is not well reported. However, ARDS in hematological malignancies carries a high 90-day mortality rate, with reported rates ranging from 45% to 73%. Here, we present a case of severe ARDS, secondary to leukemic pulmonary infiltration. Description A 72-year-old female with a past medical history significant for breast cancer (disease free following radiotherapy and chemotherapy with Taxotere Cyclophosphamide in 2013) presented with a one-month history of intermittent fevers, night sweats, unintentional weight loss and a mild productive cough. At presentation, she was hemodynamically stable, not requiring oxygen but was noted to be pancytopenic. A CT Chest/Abdomen/Pelvis demonstrated ground-glass opacities in the right middle lobe, bilateral lower lobes, along with mediastinal and hilar lymphadenopathy. Empiric antibiotics were started for a suspected pneumonia. However, bronchoscopy with bronchoalveolar lavage was unremarkable, including no evidence of Pneumocystis pneumonia. A mediastinal lymph node biopsy was also negative. To further evaluate the pancytopenia, a Bone Marrow Biopsy (BMB) was undertaken. Over the following days, the patients’ hypoxemia worsened, requiring 15 L/min of oxygen by day 14, with repeat imaging showing marked progression of the ground-glass opacities. The BMB was subsequently resulted and revealed high-grade MDS with 23% blasts, suspicious for transformation to AML. Initial criteria for concurrent Hemophagocytic Lymphohistiocytosis (HLH) was not met, but a subsequent soluble IL-2 receptor level was markedly elevated (17,675 pg/mL), suggesting evolving HLH. Due to worsening oxygen requirements, the patient was admitted to the ICU and intubated for acute hypoxemic respiratory failure secondary to ARDS (PaO2/FiO2 196). Cerebrospinal fluid analysis demonstrated atypical mononuclear cells, consistent with systemic leukemic infiltration. One day later, chemotherapy was initiated with intravenous Azacitidine and a Venetoclax ramp-up. Despite treatment, including the addition of Dexamethasone, the patient’s respiratory failure worsened, progressing to severe ARDS (PaO2/FiO2 77.6). She was subsequently transitioned to comfort care and died on day 23. Discussion High-grade MDS can present with prolonged fever and pancytopenia. Progression to AML and development of ARDS are both poor prognostic indicators. Concurrent presence of HLH in AML is rare accounting for only 6.4% of HLH cases in malignancies. Rapid deterioration in clinical status necessitates a multidisciplinary approach with consideration of pulse-dose corticosteroids. Early recognition, aimed at controlling the hyper inflammatory response and cytokine storm is linked to better survival outcomes despite the overall poor prognosis. This abstract is funded by: None
Khan et al. (Fri,) studied this question.