Blood Res.  2017 Jun;52(2):119-124. 10.5045/br.2017.52.2.119.

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

• Hwang-Bo S
• Kim SK
• Lee JW
• Jang PS
• Chung NG
• Jeong DC
• Cho B
• Kim HK

• KMID: 2413329
• DOI: http://doi.org/10.5045/br.2017.52.2.119

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.

Keyword

Autoimmune cytopenia; Autoimmune hemolytic anemia; Immune thrombocytopenia; Hematopoietic cell transplantation; Rituximab

MeSH Terms

Anemia, Hemolytic, Autoimmune
Cell Transplantation*
Child*
Diagnosis
Humans
Immunoglobulins
Incidence
Korea
Pediatrics
Purpura, Thrombocytopenic, Idiopathic
Rituximab
Transplants*
Unrelated Donors
Immunoglobulins
Rituximab

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