title: Splenectomy improves anaemia but does not reduce iron burden in patients with haemoglobin H Constant Spring disease.
abstract: BACKGROUND
  Splenectomy is reported to increase the haemoglobin level in patients with haemoglobin H Constant Spring (HbH CS) disease; however, its impact on iron burden and the underlying mechanism remains unclear.
  
  
  METHODS
  From March through to May 2013, a total of 50 adults with HbH CS disease (25 cases splenectomised and 25 cases non-splenectomised) were enrolled. The patients' general conditions, history of blood transfusion and iron chelator treatment were investigated. Levels of haemoglobin, nucleated red blood cell counts, and serum ferritin were measured. The percentage of apoptotic erythroid precursor cells in bone marrow, an index representing ineffective erythropoiesis, was determined in some cases.
  
  
  RESULTS
  There were no significant differences in age, blood transfusion volume, and use of iron chelator drugs between the splenectomised group and the non-splenectomised group. Significantly higher haemoglobin levels, serum ferritin levels and nucleated red blood cell counts as well as a higher percentage of apoptotic erythroid progenitor cells were detected in the splenectomised group. Regression analysis revealed that age and nucleated red blood cell counts were independent risk factors affecting the serum ferritin level.
  
  
  CONCLUSIONS
  Despite improving the haemoglobin level, splenectomy is associated with greater iron burden in HbH CS disease. A high nucleated red blood cell count is predictive of the risk of severe iron overload.
authors: Department of Haematology, 303rd Hospital of the People's Liberation Army, Nanning, Guangxi, China.
authors: Ya-Li
authors: YL
authors: Zhou
authors: Department of Haematology, 303rd Hospital of the People's Liberation Army, Nanning, Guangxi, China.
authors: Xin-Hua
authors: XH
authors: Zhang
authors: Department of Haematology, 303rd Hospital of the People's Liberation Army, Nanning, Guangxi, China.
authors: Tie-Niu
authors: TN
authors: Liu
authors: Department of Haematology, 303rd Hospital of the People's Liberation Army, Nanning, Guangxi, China.
authors: Li
authors: L
authors: Wang
authors: Department of Haematology, 303rd Hospital of the People's Liberation Army, Nanning, Guangxi, China.
authors: Xiao-Lin
authors: XL
authors: Yin
mesh_terms: D000328:Adult; D017209:Apoptosis; D001803:Blood Transfusion; D015672:Erythroid Precursor Cells / Q000378:metabolism* / Q000473:pathology; D005260:Female; D005293:Ferritins / Q000097:blood*; D005500:Follow-Up Studies; D006801:Humans; D007501:Iron / Q000097:blood*; D007502:Iron Chelating Agents / Q000008:administration & dosage; D008297:Male; D013156:Splenectomy; D017085:alpha-Thalassemia / Q000097:blood* / Q000473:pathology / Q000628:therapy
chemical_list: D007502:Iron Chelating Agents; D005293:Ferritins; D007501:Iron