An infant with OTT-MAL (RBM15-MKL1)-positive acute megakaryoblastic leukemia maintaining long-term remission only by induction therapy

Abstract

We report on the case of a two-month-old female with acute megakaryoblastic leukemia (AMKL), who had abdominal distension due to massive ascites. The patient was administered low-dose etoposide and cytarabine, followed by the JACLS AML 99 protocol consisting of etoposide, cytarabine, and mitoxantrone as induction therapy. She required intensive care, including mechanical ventilation for three months because of respiratory failure caused by uncontrollable massive ascites. The massive ascites was probably caused by portal hypertension due to liver fibrosis, which has been reported as one of the characteristics of OTT-MAL (or RBM15-MKL1)-positive AMKL. With hematopoietic recovery from myelosuppression, the ascites gradually improved, but we were unable to continue chemotherapy because of severe portal hypertension. However, she has maintained remission for over two years and nine months only by induction chemotherapy, and so far, her general status has been good.