Background:Acute leukemia is the most common pediatric hematological malignancy. Bloodstream infections (BSIs) are severe complications in these patients during chemotherapy. This study aimed to explore the clinical presentation and etiology of BSI, as well as the common sites of infection, and to provide a basis for the rational regarding antibiotic use.Methods:We performed a retrospective chart review of all pediatric patients who had acute leukemia accompanied by a BSI in our hospital from December 2011 to September 2015. All patients were selected based on clinical presentation and had to have at least one positive blood culture for inclusion. The basic clinical characteristics, blood culture results, and antimicrobial susceptibilities were analyzed.Results:All 231 patients had a fever; of them, 12 patients continued to have a fever. Twenty-five patients had nonremitting (NR) leukemia, and 206 patients achieved complete remission (CR). Differences in the duration of fever between the NR and CR groups were significant (9.6 ± 7.9 vs. 5.1 ± 3.8 days, P = 0.016). One hundred and eighty patients had agranulocytosis. Differences in fever duration between the agranulocytosis and nonagranulocytosis groups were significant (6.2 ± 5.1 vs. 4.1 ± 2.6 days, P = 0.001). The other sites of infection in these 231 patients were the lung, mouth, digestive tract, and rectum. Blood culture comprised 2635 samples. There were 619 samples, which were positive. Of the 619 positive blood culture samples, 59.9% had Gram-negative bacteria, 39.3% had Gram-positive bacteria, and 0.8% had fungus. The primary pathogens were Pseudomonas aeruginosa, Enterobactercloacae, Escherichia coli, and Klebsiella pneumoniae. Of these 231 patients, 217 patients were cured. The effective treatment ratio was 94%.Conclusions:Gram-negative bacteria were the main pathogenic bacteria in patients with acute leukemia in our center. NR primary illness, agranulocytosis, and drug-resistant pathogenic bacteria were all risk factors for poor prognosis.

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Objective: LPS-responsive beige-like anchor (LRBA) deficiency is one of the most common monogenic disorders causing common variable immunodeficiency (CVID) and CVID-like disorders. However, the clinical spectrum of compound heterozygous (CHZ) LRBA variation should be extended. In this study, we presented five cases of compound heterozygous LRBA with various refractory cytopenias. Materials and Methods: Retrospective analysis of the clinical manifestations, management, and outcomes of five cases (from five pedigrees) with LRBA gene CHZ variants which initially manifested as single/multilineage immune cytopenias was performed. Results: 1. Gene variations: All five patients inherited the compound heterozygous LRBA variations from their parents which were thought to be pathogenic. BEACH, DUF4704, and LamG were the main affected domains of LRBA gene in this case series. 2. Immune dysregulation of clinic: (1) Hypogammaglobulinemia were recorded in four patients, and the proportion of Treg was decreased in two patients. Only one patient had been with increased TCRαβ+CD4/CD8 double-negative T cells (DNT). (2) Lymphoproliferative manifestations were seen in three patients. (3) All five patients were complained with cytopenia, although they showed different clinical manifestations. None of the parents was asymptomatic. (4) Other immune disorders: P5 also had relapsed infections and autoimmune endocrinopathy. 3. Management and outcomes: P1 and P5 responded well to immunomodulatory therapy and P3 was effectively treated with hemophagocytic lymphohistiocytosis (HLH) first-line regimen chemotherapy. P4 showed no responses to steroids and IVIG. However, TPO-R agonist was effective. Conclusion: Unlike homozygous mutations, compound heterozygous LRBA variation should always be kept in mind for the various phenotypes and different treatment responses.