Erythroid hyperplasia of the bone marrow Specific investigations Treatment[ edit ] Plentiful literature exists regarding the treatment of AIHA. Efficacy of treatment depends on the correct diagnosis of either warm- or cold-type AIHA. Warm-type AIHA is usually a more insidious disease, not treatable by simply removing the underlying cause. Corticosteroids are first-line therapy. For those who fail to respond or have recurrent disease, splenectomy may be considered.
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Conflict-of-interest disclosure: A. All rights reserved. This article has been cited by other articles in PMC. Abstract The diagnosis of autoimmune hemolytic anemia AIHA can be made with a stepwise approach that aims to identify laboratory and clinical evidence of hemolysis and then determine the immune nature of hemolysis with the direct anti-globulin test.
Once alternative causes for these findings have been excluded, AIHA is established, and the clinician must search for secondary causes, as well as identify the type of AIHA. Rituximab is now the preferred second-line treatment for primary warm AIHA and first-line treatment for primary cold agglutinin disease CAD , either as monotherapy or combined with bendamustine. Complement inhibitors have shown utility in stabilizing AIHA patients with acute severe hemolysis.
Consequent complement activation can impact the clinical picture and is an emerging target for therapeutic approaches. When a patient presents with anemia, a stepwise approach should be followed. Initial simple investigations Table 1 will first alert the physician to the suggestion of hemolysis as the cause of the anemia. Although the typical pattern is presented, none of these tests are fully sensitive or specific for hemolysis.
For example, liver disease can increase lactate dehydrogenase LDH and reduce haptoglobin. The bilirubin may be normal with milder hemolysis, and spherocytes are not always visible.
Reticulocytopenia can occur in AIHA, secondary to bone marrow infiltration by a lymphoproliferative disorder or to parvovirus B19 infection. This may be caused by immune attack on late-stage erythroid precursors or reflect a lag in marrow responsiveness, but it can sometimes persist and predict a more severe clinical course.
There are fewer causes of intravascular hemolysis; therefore, these can be very useful for subsequent investigations Table 2. Once hemolysis is confirmed, further investigation is needed to establish whether that hemolysis is immune, principally by the direct anti-globulin test DAT.
Table 1. Modified from Hill et al, with permission.
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