Critical Care Needs and Outcome of Pediatric T cell Lymphoblastic
Leukemia or Lymphoma with Superior Venacaval Syndrome
Abstract
Background- Superior Vena Cava Syndrome (SVCS) is a
life-threatening oncological emergency affecting children with T-cell
Acute lymphoblastic Leukemia (T-ALL)/ T-Lymphoblastic Lymphoma (T-LBL).
This can prove fatal if not managed appropriately and promptly.
Method- We describe critical care needs and outcome of
T-ALL/T-LBL patients managed in our unit from 1 st May
2016 to 31 st March 2021. Result-
Twenty-three of the 120 pediatric ALL/LBL patients (19%) managed in our
unit had T-ALL/T-LBL. Eleven (48%) patients presented with SVCS. All
presented with cough and dyspnea. Chest X-Ray showed mediastinal
widening in all patients. Flow cytometry in various body fluids could
reach prompt diagnosis in 90% patients and mass biopsy was performed in
only 1 patient. Eight patients required Pediatric Intensive Care Unit
support. All 11 patients were started on corticosteroids soon after
diagnosis of SVCS. The median time to symptom relief was 4 days and that
for significant improvement on X-ray was 7days. At a median follow up of
23-months (6-63 months) overall survival and event-free survival was
75%. Conclusion- T-ALL/T-LBL patients with SVCS with good
critical care can have improved outcomes. Prompt therapeutic
interventions like pericardiocentesis can be life-saving. Flow cytometry
can be a handy tool for quick diagnosis and help avoid invasive
procedures in sick patients.