Refractory hyperhaemolysis in a patient with beta-thalassaemia major

Transfusion Medicine
J D GraingerR Wynn

Abstract

We report the case of a 1-year-old girl with newly diagnosed beta-thalassaemia major. Following an initial blood transfusion with phenotypically matched blood, she developed a haemolytic anaemia which progressed with subsequent transfusions. The Direct Antiglobulin test (DAT) was strongly positive with C3d and weakly with IgG. The only free antibodies detected were a weak anti-H and a weak cold auto-antibody, which did not exhibit a wide thermal range. The indirect Donath-Landsteiner and Ham's tests were negative. There was no sustained clinical response to steroids, immunoglobulin infusions or splenectomy. An HLA identical sibling donor was available for allogeneic bone marrow transplantation (BMT) and the haemolysis resolved during the immunosuppressive transplant conditioning. Such hyperhaemolysis without significant red cell alloantibodies has previously been reported in patients with sickle cell anaemia, but only rarely in patients with beta-thalassaemia major.

Citations

Jul 20, 2002·Biochemical and Biophysical Research Communications·Yo-ichi SuzukiTakuji Shirasawa
Jun 24, 2006·Journal of Pediatric Hematology/oncology·A KoussiP Pliaki
Dec 31, 2009·Asian Journal of Transfusion Science·Lakmali R MorawakageS K Wijewardana
Jun 25, 2014·Transfusion Medicine·A DanaeeS Robinson
Mar 28, 2006·Transfusion Medicine Reviews·Lawrence D Petz
Jun 19, 2004·Hematology·Jennifer G Treleaven, Nay Win
Mar 19, 2015·Case Reports in Hematology·Lauren Anne EberlyNathaniel Perryman Collins
Mar 22, 2012·Transfusion Medicine·G Garratty
Feb 15, 2021·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Merline AugustineSangeetha K Nayanar
Mar 6, 2010·The Medical Journal of Australia·Ian C StokesChris D Barnes

❮ Previous
Next ❯

Related Concepts

Related Feeds

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.