Wr. Drobyski et al., AUTOIMMUNE HEMOLYTIC-ANEMIA FOLLOWING T-CELL-DEPLETED ALLOGENEIC BONE-MARROW TRANSPLANTATION, Bone marrow transplantation, 17(6), 1996, pp. 1093-1099
The development of immune-mediated hemolytic anemia is a well-recogniz
ed complication after allogeneic bone marrow transplantation (BMT). Th
e majority of reported cases, however, have been alloimmune in origin
due to ABO or minor red blood cell antigen incompatibilities between t
he donor and recipient. In this study, we report seven adult patients
who developed autoimmune hemolytic anemia (AIHA) between June 1985 and
January 1993. These patients were identified from a total of 236 adul
t patients who received T cell-depleted (TCD) grafts as graft-versus-h
ost disease (GVHD) prophylaxis. The onset of AIHA was at a median of 1
0 months (range 7-25 months) post-transplant and occurred in 5% of all
patients transplanted with TCD grafts who survived at least 6 months.
Six patients had a warm reacting autoantibody, while one patient had
a cold-reacting antibody with a thermal amplitude up to 30 degrees C.
All were receiving immunosuppressive treatment for GVHD at the time of
diagnosis, Initial treatment in all patients consisted of steroids. T
hree of the seven had a partial response while the four remaining pati
ents failed to respond to corticosteroids. Splenectomy was performed i
n three patients with two partial responses. Four patients were treate
d with additional therapeutic interventions, including plasmapheresis,
immunoglobulin infusions, staphylococcus protein A column, or other i
mmunosuppressive agents. In five cases, erythropoietin was administere
d as adjunctive treatment to maintain adequate hematocrit levels. Two
patients are presently in complete remission after prolonged courses o
f steroids, while a third patient has compensated hemolysis requiring
low-dose steroids. Four patients died due to either infectious complic
ations or disseminated intravascular coagulation secondary to cold agg
lutinin disease. These data indicate that AIHA is a clinically signifi
cant and not infrequent complication in allogeneic marrow transplant r
ecipients. The response to conventional treatment is generally unsatis
factory as even patients who ultimately remit require prolonged course
s of immunosuppressive therapy.