Leukostasis-Related Fatal Cardiopulmonary Arrest as Initial Chronic Myeloid Leukemia Presentation

Abstract

Chronic myeloid leukemia (CML) is a BCR-ABL1-positive myeloproliferative neoplasm (MPN), which is usually diagnosed in chronic phase (CP-CML) and is considered curable in the era of tyrosine kinase inhibitors (TKIs). Leukostasis is an urgent complication more often of acute and less often of chronic leukemias. Regarding CML, leukostatic symptoms are uncommon in CP and in newly diagnosed patients, while being more often in advanced disease stages (accelerated or blastic phase). Leukostasis in CML is mostly presented with neurologic or respiratory symptoms. We present here a case of newly diagnosed CML in CP, which initially presented with leukostasis-related fatal cardiopulmonary arrest in a 59-year-old woman.

Fatal right‐sided heart failure due to leukostasis in a patient with leukemic transformation of myelodysplastic syndrome

“…While pulmonary involvement is a frequent complication to leukostasis, the impact of leukostasis on right ventricular function using cardiovascular imaging modalities has only been sparsely reported in the literature. Katogiannis and colleagues reported a case of leukostasis‐related fatal cardiopulmonary arrest as initial presentation of chronic myeloid leukemia with HL and a total WBC count of 390 × 10 9 /L . Similar to the present case, echocardiographic signs of severe right‐sided heart failure with preserved left ventricular function were also present in the case reported by Katogiannis and colleagues.…”

“…Katogiannis and colleagues reported a case of leukostasisrelated fatal cardiopulmonary arrest as initial presentation of chronic myeloid leukemia with HL and a total WBC count of 390 × 10 9 /L. 5 Similar to the present case, echocardiographic signs of severe right-sided heart failure with preserved left ventricular function were also present in the case reported by Katogiannis and colleagues. In the present case, the patient experienced rapid and fatal respiratory and hemodynamic deterioration within a few hours because of leukostasis despite only moderate HL.…”

Cardiovascular collapse and McConnell’s sign as early manifestations of leucostasis

“…Acute right heart failure has been less frequently described as a cause of acute cardiovascular collapse in patients with leucostasis 10 11. Katogiannis et al described the case of a 59-year-old woman who died shortly after presenting with dyspnoea for 2 days in the setting of newly diagnosed chronic myeloid leukaemia and a white blood count of 390 000/µL 10.…”

“…The most common clinical manifestations of leucostasis are neurological and respiratory symptoms, though several organ systems can be affected 3 5 6 8 9. Acute cardiovascular collapse may be an early finding 10–12…”

Blasting Into Acute Coronary Syndrome

“…In addition to causing hyperviscosity, blast cells compete aggressively with normal organ tissue for oxygen consumption. Further microvascular occlusion may be propagated by white cell plug formation [ 9 ]. In particular, patients with nonobstructive coronary artery disease are at increased risk for ischemia in the setting of blast crisis.…”