Autoimmune Hemolytic Anemia after Allogeneic Hematopoietic Stem Cell Transplantation: Analysis of 533 Adult Patients Who Underwent Transplantation at King's College Hospital

Abstract

Autoimmune hemolytic anemia (AIHA) is a recognized complication of hematopoietic stem cell transplantation (HSCT); it is often refractory to treatment and carries a high mortality. To improve understanding of the incidence, risk factors, and clinical outcome of post-transplantation AIHA, we analyzed 533 patients who received allogeneic HSCT, and we identified 19 cases of AIHA after HSCT (overall incidence, 3.6%). The median time to onset, from HSCT to AIHA, was 202 days. AIHA was associated with HSCT from unrelated donors (hazard ratio [HR], 5.28; 95% confidence interval [CI], 1.22 to 22.9; P = .026). In the majority (14 of 19; 74%) of AIHA patients, multiple agents for treatment were required, with only 9 of 19 (47%) patients achieving complete resolution of AIHA. Patients with post-transplantation AIHA had a higher overall mortality (HR, 2.48; 95% CI, 1.33 to 4.63; P = .004), with 36% (4 of 11 cases) of deaths attributable to AIHA.

Risk factor analysis of autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation in children

“…[1,2] Autoimmune hemolytic anemia (AIHA) is frequently resistant to treatment and confers decreased overall survival. [3] The clinical significance of these risk factors for developing AIHA remains uncertain, as they have not been consistently reported among studies. Traditional therapies in children with AIHA include immune modulators such as steroids or intravenous immunoglobulin (IVIG).…”

Current treatment strategies in autoimmune hemolytic disorders

“…The mortality rate of AIHA is reported to be 11% in older reports [1]; in the recent large GIMEMA study [2] mortality was~4%, and severe infections, mainly in splenectomized cases, acute renal failure, Evans' syndrome and multi-treatment (4 or more lines) were identified as predictors of a fatal outcome. Secondary forms, particularly post-transplant, are usually more severe with about a twofold increased risk of fatal outcome compared with primary AIHAs [9]. The treatment of AIHA is still not evidence-based as there are no randomized studies and only a few prospective Phase II trials [13][14][15].…”

“…Moreover, a clonal bone marrow lymphoproliferation was demonstrated in 75 and 90% of cold agglutinin disease (CAD), by bone marrow biopsy or flow cytometry, respectively [8]. As regards HSCT, AIHA occurs in about 4% of cases and is associated with clinical severity, refractoriness to therapy and poor outcome [9].…”

Blood Group Discrepancy-First Sign of Autoimmune Hemolytic Anemia after Hematopoietic Stem Cell Transplantation in a Child

“…The overall incidence of AIHA after HSCT is around 3.6 % in adults [2] and 4.4-6 % in pediatric patients [3]. There are three major risk factors associated with autoimmune manifestations in post-HSCT patients-(1) younger age of the patient, (2) diagnosis of non-malignant disease and (3) HSCT from unrelated donor or cord blood transplantation [4].…”