Autoimmune Hemolytic Anemia After Allogeneic Hematopoietic Stem Cell Transplantation In 10 Children:a Single-center Experience

Objective: To summarize the incidence, risk factors, treatment effect of autoimmune hemolytic anemia(AIHA) after allogeneic hematopoietic stem cell transplantation(HSCT) in children, and to explore the potential pathogenesis of AIHA, so as to provide reference for clinical.Methods: A retrospective analysis of the transplantation characteristics and clinical features of the patients who developed AIHA after HSCT in our center from 2007~2015 was carried out to summarize the incidence of AIHA. Related risk factors including disease, donor type, source of stem cells, conditioning regimens, GVHD prophylaxis, HLA consistency, ABO blood group, gender concordance, acute GVHD and chronic GVHD etc. was analyzed, as well as the clinical therapeutic measurements for AIHA., so as to explore the potential pathogenesis of AIHA. Chi-squared test was used for univariate comparisons ofincidence of AIHA for each clinical strata Multivariate analysis using Cox regression model was adopted. to indentify independent prognostic factors and the relation between AIHA and overall survival.Results: In 97 patients who underwent HSCT, 10 cases developed AIHA with an 1-year cumulate incidence of 5.6%;18.2% Wiskott- Aldrich syndrome(WAS)patients developed AIHA(8/44) while 41.2% patients who were concurrent with chronic graft-versus-host disease(GVHD) developed AIHA in different degree(7/17).The median time to AIHA was D+93. the cumulative incidence of AIHA in WAS patients is much higher than the group with hematopoietic malignant disease( P = 0.043). Minor mismatch of ABO blood type in donors and recipients may increase the onset of AIHA after HSCT compared with matched ones(P = 0.044).The onset of AIHA in the 10 patients were all before the fully reconstruction of immune function; while 8 patients had developed AIHA after the implantation of platelet. Chronic GVHD is an independent risk factor for AIHA after HSCT; 30% AIHA were refractory, in which 2 cases responded to rituximab treatment.Conclusions: The incidence of AIHA after allogeneic HSCT in children is relatively high, especially the WAS patients, and the incidence of AIHA in unrelated donor transplant is higher than MSD transplant; chronic GVHD may be an independent risk factor for AIHA; indicating that the profound immune dysregulation may closely relate to AIHA. Administration of rituximab may be an effective cure for intractable AIHA. A much larger, prospective study would be needed to evaluate the incidence, risk factors, pathogenetic mechanism and management of post-transplant AIHA。...