关键词: 门冬酰胺酶； 门冬酰胺酶相关性胰腺炎; 急性淋巴细胞白血病; 儿童

Abstract: Objective To analyze clinical characteristics, diagnosis, treatment and prognosis of asparaginase-associated pancreatitis (AAP) in children. Methods From January 2012 to December 2019 , 21 children with acute pancreatitis who were newly diagnosed with acute lymphoblastic leukemia (ALL) and received asparaginase-containing chemotherapy were included. Their clinical characteristics, laboratory examinations, treatment and prognosis were retrospectively analyzed. Results Among the 711 children newly diagnosed with ALL and treated with L-asparaginase (L-asp) during the study period, the incidence of AAP was 3 . 0 % (n= 21 ). Among the 21 cases, there were 10 boys and 11 girls, with a median age of 8 years (range: 3 ~ 18 years). Seven cases occurred during induction chemotherapy, after a median L-asp doses of 7 (range:1 ~ 10 doses), and 14 cases occurred in consolidation chemotherapy, with a median interval of 16 . 5 days (range:2 ~ 22 days) after PEG-asp administration, and the median number of PEG-asp doses was 4 . 5 (range:1 ~ 9 doses). Fourteen cases were classified as mild and 7 were severe. Abdominal pain and vomiting were the most common symptoms. The median values of serum amylase and lipase at AAP diagnosis were 471 U/L (range: 25 ~ 1632 U/L, upper normal limit [UNL]: 125 U/L) and 287 . 1 U/L (range: 4 . 6 ~ 2213 . 7 U/L, UNL: 31 U/L) respectively. Thirteen children underwent abdominal ultrasound examination, 6 ( 46 . 2 %) were positive; 20 cases underwent abdominal CT scan, 17 ( 85 . 0 %) were positive. Pancreatic pseudocyst occurred in 4 cases. All children received conservative management including fasting, administration of somatostatin or acid suppression drugs and nutrition support. Three cases received nasojejunal feeding, and 5 cases required surgery. Four cases with mild AAP were re-exposed to L-asp, of which one case had recurrent AAP. Except for one child who gave up treatment, there were no pancreatitis-related deaths. One case developed chronic pancreatitis and the other 19 cases were cured. Conclusion AAP is a serious complication of asparaginase therapy. Early identification is of utmost importance. The prognosis is good with multidisciplinary comprehensive treatment.

Key words: asparaginase; asparaginase-associated pancreatitis; acute lymphoblastic leukemia; child