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Biol Blood Marrow Transplant. 2008 Oct;14(10):1108-1117. doi: 10.1016/j.bbmt.2008.07.006.

Posttransplant autoimmune hemolytic anemia and other autoimmune cytopenias are increased in very young infants undergoing unrelated donor umbilical cord blood transplantation.

Author information

1
Department of Pediatrics, Division of Blood and Marrow Transplant, Duke University Medical Center, Durham, North Carolina. Electronic address: kristin.page@duke.edu.
2
The EMMES Corporation, Rockville, Maryland.
3
Department of Pediatrics, Division of Blood and Marrow Transplant, Duke University Medical Center, Durham, North Carolina.
4
Duke Human Vaccine Institute, Duke University Medical Center, Durham, North Carolina.
5
Department of Pediatrics, Division of Blood and Marrow Transplant, Duke University Medical Center, Durham, North Carolina; Department of Immunology, Duke University Medical Center, Durham, North Carolina.

Abstract

Autoimmune cytopenias are a recognized complication of hematopoietic stem cell transplant (HSCT), and are considered to be a feature of chronic graft-versus-host disease (cGVHD). We report on a cohort of very young infants (< or =3 months of age) receiving HSCT from unrelated donor umbilical cord blood for genetic disorders who developed posttransplant autoimmune cytopenias at an increased rate compared to older aged controls. These infants received a conditioning regimen consisting of busulfan, cyclophosphamide, and antithymocyte globulin (ATG). All infants received HLA mismatched unrelated umbilical cord blood as graft source. GVHD prophylaxis was either cyclosporine + methylprednisolone (n = 16) or cyclosporine + mycophenolate mofetil (n = 3). Engraftment, acute GVHD (aGVHD) and cGVHD, survival, treatment-related mortality (TRM), and deaths were evaluated. Ten patients developed cGVHD manifesting as autoimmune cytopenias at a median 247 days posttransplant with a cumulative incidence of 44% (95% confidence interval [CI] 21%-68%) and 56% (95% CI 32%-80%) at 1 and 2 years, respectively. In 6 of 10 patients developing autoimmune cytopenias, cGVHD presented as autoimmune cytopenia de novo. The cytopenias observed included anemia (n = 4), thrombocytopenia (n = 1), anemia with thrombocytopenia (n = 3), and pancytopenia (n = 2). No graft factors were identified as being significant to development of cGVHD. All patients responded to treatment with methylprednisolone, azithioprine +/- rituximab. One patient required splenectomy. We hypothesize that posttransplant immunosuppression interferes with normal immune ontogeny creating immune dysregulation and graft directed cell destruction. Alternative strategies to prevent GVHD should be considered for this unique patient population.

PMID:
18804040
PMCID:
PMC3735356
DOI:
10.1016/j.bbmt.2008.07.006
[Indexed for MEDLINE]
Free PMC Article

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