Acute autoimmune hemolytic anemia following unrelated cord blood transplantation as an early manifestation of chronic graft-versus-host disease

Abstract

Summary:A 16-month-old girl diagnosed with osteopetrosis underwent an unrelated, partially matched (with major mismatch at A locus) cord blood stem cell transplant. Twelve months later she developed severe acute autoimmune hemolytic anemia (AIHA). Immunophenotype analysis of lymphocyte subsets 8 months post transplant showed a low number of T lymphocytes, with normal subsets, and with NK cells and B lymphocytes within normal ranges. When the hemolytic anemia developed, the lymphocytes subsets changed and analysis showed higher numbers of B lymphocytes than previously, lower CD3+ T lymphocytes with inversion of the CD4/CD8 ratio and an abnormal proportion of T lymphocyte subsets. She was being treated with cyclosporine, and steroids and immunoglobulins were added. Initially the AIHA improved, but repeated infectious episodes led us to tail off the immunosuppressive treatment. The AIHA relapsed and cyclosporine was restarted. Currently, she is on cyclosporine and lowdose steroid treatment with no hemolytic features. During the 3 months when the AIHA was being treated, she developed extensive skin cGVHD and recurrent pneumothoraces. AIHA may be the first manifestation of abnormal reconstitution of immunity developing after a hematopoietic transplant. This abnormal reconstitution is also the basis of cGVHD. We suggest that aggressive immunosuppressive treatment with intensive measures against infection could give a better prognosis to such patients. Bone Marrow Transplantation (2001) 28, 89-92.

Autoimmune hemolytic anemia following allogeneic hematopoietic stem cell transplantation in adult patients

“…[25][26][27][28] In fact, AIHA can even be a presenting sign of cGVHD. 8,29 The mechanism by which allogeneic cellular activation leads to a humoral autoimmune disorder is unclear. It has also been suggested that CyA could impair development of immune tolerance in the post-transplant period and thus induce the development of autoimmune reactions.…”

“…5 However, the incidence and risk factors for the development of AIHA, as well as its prognosis and response to treatment are still not well defined. In fact, our current knowledge of AIHA is based on single case reports [6][7][8][9] and small series in adults 4,10 and children. 5 The aim of the study was to analyze the characteristics of patients and transplants, as well as outcome, in a series of patients who developed AIHA after allogeneic HSCT at a single institution.…”

Evans Syndrome After Unrelated Cord Blood Transplantation for Disseminated Langerhans Cell Histiocytosis in a Child

“…A case report of AIHA after CBT for osteopetrosis also showed a low number of T lymphocytes by immunophenotype analysis. 12 Indeed, unbalanced B and T lymphopoiesis has been proposed to favor the development of the antired blood cell antibodies causing AIHA. 9 In addition, in vivo T-cell depletion by antihuman thymocyte globulin in our patient might have played a role in the development of Evans syndrome.…”

Immune hemolytic anemia—selected topics

“…Additional reports have included several cases following cord blood transplants, with resultant AIHA and Evans syndrome. 25,26 There have been reports of successful therapy of AIHA following hematopoietic stem cell transplantation with rituximab.…”