Childhood Acute Leukemia with Unfavorable Cytogenetics: A Case of Monosomy 7

Purpose: Though not as common as acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML) represents a significant malignancy burden in pediatric populations. Survival rates are climbing as research improves our understanding of the disease process, but survival in AML remains below that of other childhood cancers. The purpose of this study was to examine a case of AML with unfavorable cytogenetics in the leukemia cells (monosomy 7) and observe the barriers to success in a high-risk patient. Methods: A recently diagnosed, 6-year old Hispanic male patient at Cook Children’s Medical Center was chosen for study because of his high-risk status due to monosomy 7 and challenging course of treatment and management. Medical records were reviewed for the entire course of his treatment, from diagnosis to eventual death. As the records were reviewed, special attention was paid to disease progression, resolution (or lack thereof) of risk factors, and development of treatment-related complications. Results: During the course of his treatment, the patient was diagnosed with monosomy 7-positive AML with central nervous system (CNS) infiltration. He endured two courses of intensive induction chemotherapy and underwent two unrelated cord blood stem cell transplants. Both transplants ultimately resulted in primary engraftment failure, and the second was followed by clinical deterioration and death. During the course of therapy, the patient suffered from severe treatment-related immunosuppression that enhanced his risk for a variety of infectious complications. Despite aggressive interventions by the medical team including 82 days in the intensive care unit, the patient’s status declined to irreversible multi-organ failure leading to the implementation of a palliative approach and the parents’ decision to withdraw aggressive life support. The patient died from complications of persistent bone marrow failure having failed to achieve hematopoietic recovery despite two stem cell transplants. Conclusions: Unfortunately, failure of treatment is not uncommon in patients with high-risk AML. The combination of monosomy 7 and CNS leukemia infiltration provided a poor prognosis at the time of diagnosis, and the patient was unable to overcome the complications of his disease and aggressive treatment course. New approaches to therapy based upon molecular targets and/or immune-based strategies are needed to offer a better prospect of survival for patients with high risk AML.