BackgroundAutoimmune cytopenia (AIC) is a rare complication of allogeneic hematopoietic cell transplantation (HCT). In this study, we reviewed the diagnosis, treatment and response to therapy for pediatric patients with post-HCT AIC at our institution.MethodsOf the 292 allogeneic HCTs performed from January, 2011 to December, 2015 at the Department of Pediatrics, The Catholic University of Korea, seven were complicated by post-HCT AIC, resulting in an incidence of 2.4%.ResultsAll seven patients with post-HCT AIC had received unrelated donor transplant. Six of seven patients had a major donor-recipient blood type mismatch. The subtypes of AIC were as follows: immune thrombocytopenia (ITP) 2, autoimmune hemolytic anemia (AIHA) 2, Evans syndrome 3. Median time from HCT to AIC diagnosis was 3.6 months. All but one patient responded to first line therapy of steroid±intravenous immunoglobulin (IVIG), but none achieved complete response (CR) with this treatment. After a median duration of treatment of 15.3 months, two patients with ITP achieved CR and five had partial response (PR) of AIC. Five patients were treated with rituximab, resulting in the following response: 2 CR, 2 PR, 1 no response (NR). Median time to response to rituximab was 26 days from first infusion. All patients are alive without event.ConclusionPost-HCT AIC is a rare complication that may not resolve despite prolonged therapy. Rapid initiation of second line agents including but not limited to B cell depleting treatment should be considered for those that fail to achieve CR with first line therapy.

Epiploic appendagitis is an inflammation of the epiploic appendage in which the small sacs projecting from the serosal layer of the colon are positioned longitudinally from the caecum to the rectosigmoid area. Epiploic appendagitis is rare and self-limiting; however, it can cause sudden abdominal pain in children. Epiploic appendagitis does not typically accompany other gastrointestinal diseases. Here, we report on a healthy eight-year-old girl who presented with abdominal pain, fever, vomiting, and diarrhea. Based on these symptoms, she was diagnosed with acute gastroenteritis, but epiploic appendagitis in the ascending colon was revealed in contrast computed tomography (CT). The patient was treated successfully with conservative management. CT is beneficial in diagnosis and further assessment of epiploic appendagitis. Pediatricians need to be aware of this self-limiting disease and consider it as a possible alternate diagnosis in cases of acute abdominal pain.

Background:Although adenovirus (ADV) infection usually causes self-limiting respiratory disorders in immune competent children; severe and systemic ADV infection in children undergoing chemotherapy for leukemia has been continuously reported. Nevertheless, there has been no consensus on risk factors and treatment strategies for severe ADV infection in children undergoing chemotherapy.Case summary:We report a case of a 15-year-old boy with a fatal systemic ADV infection. He had received reinduction chemotherapy for relapsed acute lymphoblastic leukemia under continuing antifungal therapy for previously diagnosed fungal pneumonia. He complained of fever and right shoulder pain 4 days after completing the reinduction chemotherapy. In spite of appropriate antibiotic and antifungal therapy, pneumonia was aggravated and gross hematuria was accompanied. A multiplex polymerase chain reaction test for respiratory viruses was positive for ADV in a blood sample, and a urine culture was positive for ADV. He received oral ribavirin, intravenous immunoglobulin, and intravenous cidofovir therapy; however, he eventually died. Relapsed leukemia, concurrent fungal pneumonia, and delayed cidofovir administration were considered the cause of the grave outcome in this patient.Conclusion:ADV may cause severe infections not only in allogeneic hematopoietic cell transplant recipients, but also in patients undergoing chemotherapy for acute leukemia. The risk factors for severe ADV infection in patients undergoing chemotherapy should be determined in the future studies, and early antiviral therapy should be administered to immune compromised patients with systemic ADV infection.

The aim was to examine whether clinical seizures and amplitude-integrated electroencephalogram (aEEG) patterns in infants with hypoxic ischemic encephalopathy (HIE) can predict the extent of brain injury on magnetic resonance images (MRI) and the long-term neurodevelopmental outcomes at 18∼24 months of age. HIE infants who underwent therapeutic hypothermia (TH) between June 2014 and March 2017 were included in this study. Infants with clinical seizure were analyzed for aEEG patterns and the extent of brain injury on MRI findings. Clinical seizure, aEEG, and brain MRI were assessed and compared with neurodevelopmental outcomes at 18∼24 months of age. Among the 97 HIE infants enrolled in this study with brain MRI scans, 78 (73.1%) TH-treated HIE infants exhibited clinical seizures. More abnormalities on a EEGs and more significant use of first and second antiepileptic drugs (AEDs) were significantly higher in the clinical-seizure group with longer hospitalized days. At a corrected 18 to 24 months of age, HIE infants in the clinical-seizure group with more extension of injury lesions on diffusion-weighted MRI scans exhibited significantly more delayed neurodevelopment. A risk factor analysis indicated that male infants who stayed in the hospital for more than 11 days were at a higher risk of having clinical seizures. The lesion size in MRI greater than 37 pixels was a risk factor with an 81.8% accuracy. Seizures in HIE infants may predict abnormal brain MRI scans and abnormal neurodevelopment at 18 to 24 months of age.