Summary:would be expected for non-BMT patients. The patients reported here fall into two groups, an early onset group associated with cold autoantibodies and a late onset group We reviewed the medical records of 293 patients who underwent allogeneic bone marrow transplants at the associated with warm antibodies. In the early group the antibodies occurred between 2 to 8 months and in the late Hammersmith Hospital between 1989 and 1994. There was clinical evidence of an autoimmune reaction against onset group between 6 and 18 months post-transplant. We discuss the relationship between these phenomena and the red cells in nine patients. Seven of these patients had significant haemolysis; the other two had red cell autorole of factors such as GVHD, relapse, CMV and immunosuppression and also the association with the regenerating agglutination. Haemolysis was resistant to treatment in three cases. Six of the nine patients had monoclonal Ig immune system following BMT. bands identified within 1 year of transplant. The autoimmune reaction could be classified broadly into two types: an early onset type (n = 4) beginning 2 to 8Patients and methods months post-transplant associated with a cold antibody, and a late onset type (n = 5) beginning 6 to 18 months From the 293 patients who underwent allogeneic BMT at post-transplant associated with warm antibodies. The the Hammersmith Hospital, we identified nine patients with predominant antibody in the two categories described documented anti-red cell autoantibodies (red cell autoimmay reflect the kinetics of immune reconstitution postmunisation, RCAI). These patients were identified because transplant, since serum IgM levels typically return to they had clinically evident haemolysis (falling Hb with normal 2 to 6 months post-transplant, while IgG levels elevated bilirubin and reticulocytosis) or blood film abnormay not reach normal levels until 12-18 months postmality (eg agglutination). Patient and BMT details are transplant. We speculate that unbalanced reconstitution shown in Table 1. Two donor-recipient pairs had minor of B and T cell lymphopoiesis post-transplant may fav-ABO mismatch, one a major and one a bidirectional ABO our emergence of oligoclonal proliferation and that mismatch. All were matched for Rh D type. Haemolytic some of the resulting antibodies may have activity episodes attributable to ABO and Rh incompatibility are against red cells. not included in this report. One of the cases (No. 4) has
A 17-year-old boy developed autoimmune pancytopenia in the absence of chronic graft-versus-host disease 170 d after allogeneic bone marrow transplantation (BMT) from his HLA identical brother. The anaemia and thrombocytopenia responded to conventional immunosuppressive treatment, but the neutropenia was refractory to this and to splenectomy and subsequent removal of splenic remnant. Following total lymphoid irradiation the neutrophil count rose to low normal levels but thrombocytopenia and anaemia secondary to marrow hypoplasia required transfusion support. Bone marrow function was finally normalized by an additional transfusion of donor marrow without prior immunosuppressive therapy. We conclude that late onset immune pancytopenia post BMT caused by antibodies of probable donor origin may be life threatening in the absence of chronic graft-versus-host disease.
A patient presented with haemolytic anaemia and a negative direct antiglobulin test (DAT), and was found to have an IgG antibody with anti-Jka specificity in his serum. His red cells were typed as Jk(a-b+). Later he developed idiopathic thrombocytopenic purpura (ITP), and had a positive DAT due to anti-Jka bound to his red cells, which now typed as Jk(a+b+). Family studies suggested that the patient's true type was Jk(a+b+). Splenectomy and immunosuppression were required to treat the thrombocytopenia. The autoanti-Jka was no longer detectable following therapy.
A case of autoimmune hemolytic anemia (AIHA) in a young child is described. The hemolysis was resistant to steroid therapy but responded to splenectomy and intravenous immunoglobulin. The autoantibody was shown to be anti-Sc1 by both serologic and immunoblotting techniques. This seems to be the first report of an autoanti-Sc1 detected by immunoblotting and the first example of AIHA in a child caused by autoanti-Sc1.
The use of the direct manual hexadimethrine bromide (Polybrene) test (DPT) in the investigation of patients for autoimmune hemolytic anemia (AIHA) was evaluated. Seventy-nine blood samples from 68 patients were tested. A direct antiglobulin test (DAT) using monospecific reagents and the DPT were performed, and a concentrated ether eluate was tested. The DAT was positive in 62 (78%) of 79 patients and negative in 17 (22%). There is a good correlation among DAT, eluate, and DPT in demonstrating the presence of immunoglobulin on the red cell surface. In contrast, the DPT does not detect C3d and is often negative in cases of AIHA in which C3d alone is demonstrated by the DAT. In DAT-negative cases, DPT results correlated with reactive eluates. However, in four cases of steroid-responsive, DAT-negative hemolytic anemia, the DPT supported the diagnosis of AIHA when the eluate did not react. The DPT is a useful additional screening test for the investigation of AIHA, but it is not recommended as a replacement for either eluate testing or the DAT.
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