Blood Res 2017; 52(2): 119-124  https://doi.org/10.5045/br.2017.52.2.119
Treatment and response of autoimmune cytopenia occurring after allogeneic hematopoietic cell transplantation in children
Seok Hwang-Bo, Seong-koo Kim, Jae Wook Lee, Pil-Sang Jang, Nack-Gyun Chung, Dae-Chul Jeong, Bin Cho, and Hack-Ki Kim

Division of Hematology and Oncology, Department of Pediatrics, The Catholic University of Korea, Seoul, Korea.

Correspondence to: Bin Cho, M.D., Ph.D. Department of Pediatrics, Seoul Saint Mary's Hospital, The Catholic University of Korea, Banpo-daero 222, Seocho-gu, Seoul 06591, Korea. chobinkr@catholic.ac.kr
Received: February 23, 2017; Revised: March 30, 2017; Accepted: April 11, 2017; Published online: June 22, 2017.
© The Korean Journal of Hematology. All rights reserved.

Abstract

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.

Keywords: Autoimmune cytopenia, Autoimmune hemolytic anemia, Immune thrombocytopenia, Hematopoietic cell transplantation, Rituximab


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