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Objective To analyze the distribution and drug resistance of pathogenic bacteria in blood- stream infection inpediatric hematology department, providing references of clinical empirical medication. Methods In this study, we retrospectively analyzed the BSI in children with hematological diseases from 2016 to 2021 at the second hospital of Anhui Medical University, including an assessment of the clinical characteristics, distribution of pathogens, and antibiotic resistance data. Results There were 145 patients with BSI, 155 strains of pathogenic were isolated from the patients, including 92 strains (59.3%) of gram-negative bacteria and 59strains (38.1%) of gram-positive bacteria. The top four proportion of pathogenic bacteria were coagulase - negative staphylococcus (20%), Klebsiella pneumonia (16.1%), Escherichia Coli (13.5%), and Pseudomonas aeruginosa (9.7%). The proportion of Pseudomonas aeruginosa increased gradually. The patients of acute myeloid leukemia (AML) were more likely to be infected with gram-positive bacteria than than acute lymphoblastic leukemia (ALL). There were 66.7% and 40.0% Klebsiella pneumonia and Escherichia Coli with extended-spectrum β-lactamase-producing respectively. Susceptibility rates of Klebsiella pneumonia and Escherichia Coli to carbapenems were 88.0% and 100.0%. Resistance rates of Pseudomonas aeruginosa to imipenem and meropenem were 26.6% and 13.3% respectively. Detection rate of Methicillin - resistant coagulase - negative staphylococci (MRCNS) and Methicillin - resistant staphylococcus aureus (MRSA) were 81.1% and 32.4% respectively. No bacteria with vancomycin-resistant and linezolid-resistant were found in staphylococcus. Conclusions The main pathogenic bacteria of BSI hospitalized in the pediatric hematology department is gram - negative bacteria, proportion of some species has increasing tendency. The patients of AML are more likely to be infected with gram-positive bacteria than the patients of ALL. Antibiotics should be selected rationally based on the diseases, distribution of pathogens and drug resistance. [ABSTRACT FROM AUTHOR]