Autoimmune Hematological Diseases Following Haploidentical Donor Hematopoietic Stem Cell Transplant Compared with Matched Sibling and Unrelated Donor

Objective:Autoimmune hematological diseases (AHDs) occur more frequently than other autoimmune complications after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and are often refractory to treatment. The aim of this study was to analyze the incidence and risk factors of AHDs as well as its response to treatment in a series of patients undergoing allo-HSCT at our single institution.

Method:Forty hundred and forty-five adult malignant hematopoietic disorders underwent allo-HSCT were enrolled in this retrospective study, including 181 cases underwent HLA-matched sibling donor (MSD) transplants, 140 unrelated donor (MUD) transplants and 124 haploidentical donor transplants (HRD).

Result:Twelve patients developed AHDs, including 6 autoimmune hemolytic anemia (AIHA)and 6 Evans' syndrome.The median time of AHDs onset was 196 days (range, 60-756). Evans' syndrome all ocuured in HRD. The 3-year cumulative incidence of AHDs was 2.7%,and HRD had higher incidence than MSD (6.5% vs 1.1%,P=0.017) and MUD(6.5%vs1.4%,P=0.049). Eight of them received complete response (CR) and 3 no response (NR) after 11 patients received the corticosteroids combined with CSA as the initial treatment for 4 weeks. One obtained CR and 2 PR after 3 NR cases treated by rituximab. Eleven cases all not were AHDs recurrence at a median followup of 313 days (range, 153 to 1051 days after CR. HRD transplantation(vs MSD: hazard ratio [HR], 7.70; 95% confidence interval [CI], 1.63 to 36.44; P=.01 and vs MUD: hazard ratio [HR], 5.87; 95% confidence interval [CI], 1.24 to 27.73; P=.026) and concurrent chronic GVHD (hazard ratio [HR], 3.76; 95% confidence interval [CI], 1.18 to 11.92; P=.025)were risk factors for AHDs.

Conclusion:The patients undergoing HRD transplantation had higher risk of AHDs and manifested as Evan's syndrome was very common compared with MSD and MUD. Corticosteroids combined with CSA as the first line treatment of AHDs results in good response and low recurrence rate.