Transfusion independence after repeated haploidentical hematopoietic cell transplants in a patient with congenital dyserythropoietic anemia type II and hemosiderosis

Pediatr Transplant. 2019 Dec;23(8):e13587. doi: 10.1111/petr.13587. Epub 2019 Sep 17.

Abstract

Matched related or unrelated donor allogeneic HCT has occasionally been applied in patients with severe CDA type II and proven to be curative. We report on the first patient with CDA to undergo haploidentical bone marrow transplantation with PT-CY. A 12-year-old boy with severe hemosiderosis, and a, consequently, disturbed BM microenvironment, developed recurrent graft failures and required salvage with two additional haploidentical HCTs. He achieved complete donor chimerism and transfusion independence after the third HCT. Our case underscores the risks associated with performing haploidentical HCT in older pediatric patients with CDA and severe chronic iron overload.

Keywords: congenital dyserythropoietic anemias; graft failure; haploidentical HCT.

Publication types

  • Case Reports

MeSH terms

  • Anemia, Dyserythropoietic, Congenital / complications
  • Anemia, Dyserythropoietic, Congenital / surgery*
  • Blood Transfusion
  • Child
  • Hematopoietic Stem Cell Transplantation* / methods
  • Hemosiderosis / complications
  • Humans
  • Male
  • Severity of Illness Index
  • Transplantation, Haploidentical