Patients with AIHA who did not require new AIHA-directed pharmacologic treatment
Seventeen patients were managed without modification of their current therapy and received supportive care only for their AIHA. Of these 17 patients, 15 patients (88%) were on immunosuppression at the time of AIHA diagnosis, described further below. Three patients had a history of an immune cytopenia prior to transplant including immune thrombocytopenia (n=1) and AIHA (n=2). These patients were significantly older at the time of transplant (median 10.02 years, range: 0.22-22.71) compared to those treated with corticosteroids-only (median 0.46 years, range: 0.14-3.88) and those treated with more than one AIHA-directed pharmacologic (median 4.98 years, range 0.71-12.91); p=0.009 (Table 3). AIHA occurred at a median of 2.83 months (range: 0.95-12.88) after HSCT in this group in comparison to 5.91 months (range: 3.71-29.73) in the corticosteroid-only treated group and 4.72 months (range 3.19-7.43) in those treated with more than one AIHA-directed treatment. Similar to the corticosteroid-treated group, most (n=15) had a BM donor source, 1 had a peripheral blood source, and 1 had a cord blood source. Most patients in this group had a MURD (n=10). Five patients (29.4%) had malignant disease as compared with only 2 patients (12.5%) in the entire pharmacologic treatment group. Four patients had acute GVHD in the first year after transplant and five patients had chronic GVHD. Five patients (29.4%) had CMV infection and 3 (17.6%) had EBV infection concurrently or prior to AIHA after transplant.
In this group of 17 patients, the median hemoglobin at time of AIHA diagnosis was 7.7 g/dL (range: 5.6-10.8) compared to 6.4 g/dL (range 4-10) in the corticosteroid-only group and 6.8 g/dL in the group treated with more than one AIHA-directed pharmacologic (p=0.07). Only ten patients (58.8%) in this group received a pRBC transfusion in the first two weeks after AIHA diagnosis compared to 100% in the corticosteroid-only group and 85.7% in the group treated with more than one AIHA-directed pharmacologic (p=0.15). Although 9 of these patients (52.9%) were already admitted at the time of AIHA diagnosis, no patient in this group required a new admission for management of AIHA.
In this group that did not receive new AIHA-directed treatment, 15 patients (88%) were on immunosuppression at the time of AIHA diagnosis. Eight of the 15 patients (53%) were on immunosuppression for prophylaxis of GVHD including cyclosporine, tacrolimus, and mycophenolate; 4 of these patients were also on a corticosteroid taper. Four of the 15 patients were on immunosuppression for treatment of acute GVHD; 2 patients were receiving both cyclosporine and biologics (infliximab and basilixumab) and weaning corticosteroids; 1 patient was receiving cyclosporine and weaning corticosteroids, and 1 patient was on corticosteroids alone. The 3 remaining patients managed with supportive care were on immunosuppressives for other reasons (concern for relapsed leukemia, treatment of nephropathy, and treatment of neutropenia and thrombocytopenia). Two patients were not on immunosuppression.
After one month of observation, 7 patients had a complete response of their AIHA, 7 patients had a partial response, and 1 patient had non-response (unknown, n=2). Longer term follow-up and remission status was more difficult to obtain in this group due to relapsed disease, development of post-transplant lymphoproliferative disease, death due to other causes, need for a second transplant, and patient re-location. It is not evident that AIHA was a contributor to these outcomes.