Treatment and response of autoimmune cytopenia occurring after allogeneic hematopoietic cell transplantation in children

BACKGROUND: Autoimmune 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. METHODS: Of 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%. RESULTS: All 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. CONCLUSION: Post-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.

A Case of Epiploic Appendagitis with Acute Gastroenteritis / 대한소아소화기영양학회지

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.