The Urgent Management of T-Cell Acute Lymphoblastic Leukemia (T-ALL) in Children

The Urgent Management of T-Cell Acute Lymphoblastic Leukemia (T-ALL) in Children Purpose: Children with T-ALL often have aggressive disease with life-threatening problems at the time of diagnosis. This presentation of three cases describes the diagnostic process of T-ALL, discusses the clinical options involved in initial management, and delineates expectations for patients prior to discharge. This descriptive study highlights the most important steps in managing a patient with T-ALL and its associated complications. Method: Three recently diagnosed cases (late 2015) were available for study. Patient records were reviewed to gather information regarding history, physical exam, laboratory data, imaging, and management. Results: Each of these patients, ages 18 months, 3 years, and 10 years of age, had a different clinical presentation. They were referred to Cook Children’s Medical Center (CCMC) after evaluation by a primary care or urgent care provider. The suspicion of leukemia was confirmed with peripheral blood smears and flow cytometry. Two of the three patients had a mediastinal mass present. All patients presented with severe leukocytosis with white blood cell counts ranging from 425,000 to 781,000/mm3. Due to the concerns of leukostasis, they each underwent leukapheresis to decrease their white cell counts. Concerns addressed during admission included respiratory distress, cerebral leukostasis, and tumor lysis syndrome. These patients spent an average of eleven days in the hospital with each patient spending five days in the Pediatric Intensive Care Unit. Two were enrolled in a randomized clinical trial assessing the efficacy of investigational drug bortezomib in T-ALL. The patient that was not enrolled in the trial received the current standard of care. Conclusion: T-ALL (with or without mediastinal mass) can be the underlying cause of life threatening clinical problems. Although protocols are the backbone of treatment, there may be differences in the initial management, including the decision to utilize leukapheresis to bring WBC levels from initially dangerous elevations to values that are safer before beginning anti-leukemic therapy, as well as steroid preconditioning. Patients were discharged after evidence of clinical stability, treatment progress, and improvement in hematological lab values. In addition, each of these patients had family demonstrating commitment to follow up for additional treatment.