Exocrine pancreatic insufficiency following asparaginase-induced pancreatitis in pediatric acute lymphoblastic leukemia patients: A case report




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Background: Asparaginase is an essential agent in the treatment of acute lymphoblastic leukemia (ALL) in pediatric patients. Despite its clinical benefits, patients are at risk for adverse effects including but not limited to asparaginase-associated pancreatitis (AAP). Exocrine pancreatic insufficiency (EPI) is a related condition that involves impaired secretion of digestive enzymes. Case presentations: A 14-year-old male with T cell lymphoblastic leukemia was tolerating chemotherapy well until day 21 of induction therapy when he was brought to the emergency room for generalized weakness and severe abdominal pain. Notably, his appetite had decreased in association with the abdominal pain. Due to his symptoms and recent PEG asparaginase administration, serum lipase was evaluated. Additionally, lipase was evaluated at 1371 U/L, so he was diagnosed with pancreatitis and initially made NPO. Weeks later, he had ongoing difficulties with weight gain and given that his abdominal pain and lipase had improved, the diet was liberalized to a regular diet. Difficulty with adequate intake and weight loss persisted, prompting evaluation of fecal elastase. The results showed a fecal elastase level of less than 10 (normal >/= 201 ug/g), indicating severe exocrine pancreatic insufficiency. The patient was started on pancreatic enzyme replacement therapy (PERT). Fecal elastase was repeated 4 months after EPI diagnosis. While the level had improved slightly (32 ug/g), it still indicated severe pancreatic insufficiency. Five months later, the fecal elastase was re-evaluated again, and it had improved to 131 ug/g, indicating moderate pancreatic insufficiency. The family was instructed to reduce PERT dosing by half with lower fat meals and snacks while monitoring for recurrence of malabsorption symptoms. The patient remained on the lower PERT dosing for three months until he began to have weight loss, abdominal pain, and intermittent loose stools. He was due for an updated fecal elastase level, which had reduced to 85 ug/g. Given the patient’s symptoms and low fecal elastase, PERT was increased to previous dosing using 24,000 UL/capsule. A 6-year-old female patient with preexisting obesity and recently diagnosed Pre-B acute lymphoblastic leukemia presented to the emergency room on day 12 of induction chemotherapy with complaints of severe abdominal pain, nausea, and vomiting. Her symptoms and recent PEG asparaginase administration, warranted serum lipase evaluation. She was diagnosed with pancreatitis due to her supporting symptoms and elevated lipase. Her nutritional intake improved, but the patient continued to experience diarrhea and abdominal pain, prompting evaluation of fecal elastase. Fecal elastase level was 22 ug/g, and PERT was started. Following a week on pancreatic enzyme replacement, the patient’s oral intake had increased. The patient had no complaints of oily stools despite not taking enzymes. She was able to trial off enzyme replacement therapy Conclusion: EPI is an important and often undiagnosed clinical condition with potential deleterious effects on the nutritional status of patients with pancreatitis. It is critical to evaluate for signs of malabsorption, such as oily stools, weight loss, abdominal pain, and bloating in patients with AAP.