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.