Abstract / Summary
Pure erythroid leukemia (PEL) is an extremely rare subtype of acute myeloid leukemia (AML), accounting for only 1% of cases, marked by complex karyotypes, dismal prognosis, and lack of standardized treatment. This study reports four cases of PEL with rapidly progressive clinical manifestations, including fever, marked conjugated hyperbilirubinemia, extreme elevations of lactate dehydrogenase (LDH) and ferritin, splenomegaly, ascites, and hepatic failure. Malignant erythroid infiltration of the hepatic sinusoids may be a pathological mechanism responsible for acute liver failure in PEL. Among the four patients, three died of liver failure, while one achieved sustained remission after early diagnosis and intensive multi-agent chemotherapy. Overall, this study highlights the urgency of early identification of this fatal PEL subtype, clarifies the mechanisms underlying its jaundice and hepatic failure, and emphasizes timely intervention to improve patient outcomes.