From the Guidelines
No improvement in hemoglobin levels despite repeated blood transfusions can be caused by hyperhemolysis, a condition where the transfused red blood cells are rapidly destroyed, often due to immune-mediated mechanisms, as highlighted in the 2020 American Society of Hematology guidelines for sickle cell disease: transfusion support 1. This condition can lead to a rapid decline in hemoglobin levels, despite repeated transfusions, and is a life-threatening complication that requires prompt recognition and treatment. Other factors that can contribute to no improvement in hemoglobin levels include ongoing blood loss, hemolysis, hypersplenism, bone marrow suppression, and antibody-mediated destruction of transfused cells. Key considerations in the management of hyperhemolysis include:
- Avoiding further transfusions unless absolutely necessary, as they can exacerbate the condition 1
- Using extended matched red cells that lack the offending antigen to reduce the risk of immune-mediated destruction 1
- Initiating immunosuppressive therapy, such as IVIg, high-dose steroids, or eculizumab, to reduce the immune response and prevent further hemolysis 1
- Providing supportive care, including erythropoietin and IV iron, to help improve hemoglobin levels and reduce the need for transfusions 1 It is essential to identify and address the underlying cause of the lack of improvement in hemoglobin levels, rather than simply continuing transfusions, to prevent further complications and improve patient outcomes.
From the Research
Causes of No Improvement in Hemoglobin Levels
Despite repeated blood transfusions, there are several factors that can contribute to no improvement in hemoglobin (Hb) levels. These include:
- Functional iron deficiency, which can rapidly ensue when erythropboietin is used to treat anemias, unless individuals are iron-overloaded from prior transfusions 2
- Inadequate iron therapy, which is essential to maximize erythropboiesis and avoid absolute and functional iron deficiency 2
- Severe autoimmune hemolytic anemia (AIHA), where repeated blood transfusions may be unsuccessful in maintaining safe hemoglobin levels 3
- Chronic anemia, where the use of erythropboietin stimulating agents may be necessary to improve hemoglobin levels 4, 5
- Intercurrent and/or unpredictable events, such as hospitalization, blood transfusion, and laboratory error or misreported hemoglobin values, which can affect the accuracy of predictive models for hemoglobin response to darbepoetin and iron administration 5
Factors Affecting Hemoglobin Response
Several factors can affect the response of hemoglobin levels to treatment, including:
- The type and dose of erythropboietin stimulating agents used 6
- The presence of comorbidities, such as cardiovascular disease or kidney failure 4, 6
- The use of iron supplementation and the maintenance of adequate body iron stores 2, 5
- The accuracy of hemoglobin measurements and the presence of laboratory errors or misreported values 5