Clinical characteristics of patients with AIHA
In patients with AIHA, the median age at time of transplant was 7.4
years (range: 0.1-23.2) and median time to development of AIHA after the
day of transplant was 4.7 months (range: 1.0-29.7). At the time of AIHA
diagnosis, all patients had a positive direct antiglobulin test,
including IgG and C3 (n=16), IgG only (n=11), C3 only (n=4), and two
patients in which neither IgG nor C3 was identified. IgM, IgA, and
Donath-Landsteiner antibodies were not tested. Fifteen patients (15/33,
45.5%) converted their blood type from recipient to donor prior to the
time of AIHA diagnosis, 6 converted after AIHA diagnosis, 8
recipient/donor pairs had identical blood types, and, in 4 patients,
time to conversion was unable to be determined. Sixty-nine percent of
patients (20/29, unknown = 4) had >28 days between their
last packed red blood cell transfusion (pRBC) and onset of AIHA. The
median number of days between last pRBC transfusion and development of
AIHA was 73.5 days (range: 1-889).
CMV and EBV infections occurred either prior to or concurrently with
AIHA diagnosis in a portion of patients with AIHA. CMV infection
occurred in 24.2% (8/33) of patients with AIHA; of those with an
infection, 62.5% (5/8) had positive blood CMV PCR testing at the time
of diagnosis of AIHA. EBV infection occurred in 21.2% (7/33) with AIHA,
and, of those with an infection, 100% (7/7) had positive blood EBV PCR
testing at the time of AIHA. GVHD was diagnosed in a minority of
patients with AIHA; 7 patients (21.2%) had acute GVHD in the first 100
days after transplant (grade range 1-3). Two patients required GVHD
treatment with systemic steroid monotherapy and 4 patients required
combination therapy with systemic corticosteroids and additional agents.
Eight patients (24.2%) had chronic GVHD in the first-year after
transplant with 1 patient (12.5%) having limited disease and 7 patients
(87.5%) having extensive disease. Seven of these patients required
treatment with systemic corticosteroids and additional agents.