Autoimmune hemolytic anemia is a rare disorder in childhood. The therapeutical difficulties encountered are described in a series of 14 patients aged 6 weeks to 10 years, 8 of them being under the age of 1 year at time of diagnosis. A remission was observed spontaneously in 1 patient and was obtained with steroids in 8 others. One patient died from acute irreversible hemolysis. Four patients were splenectomized because of an immediate or delayed resistance to steroids. After splenectomy, two patients died from infection, one was cured and the last patient is still in remission after one year. Steroid therapy is the primary treatment of autoimmune hemolytic anemia. The initial dose of 2 mg/kg/day has to be maintained until the remission (normal hemoglobin level and reticulocyte count) is achieved, then progressively decreased leading to alternate day therapy for several months. Resistance to steroid requires splenectomy, although it is an hazardous treatment in young children. The indication for immunosuppressive agents in childhood is difficult to define. It can be proposed in patients with steroid dependency in order to reduce the dose of steroid and/or to avoid long term steroid therapy.
The diagnosis of hemolytic anemia can be suspected on the basis of a constellation of symptoms and is largely based on examination of a peripheral blood smear and a number of laboratory studies. Symptoms of hemolytic anemia include those that can occur in all anemias as well as the specific consequences of hemolysis. All anemias can cause fatigue, shortness of breath, decreased ability to exercise when severe. Symptoms specifically related to hemolysis include jaundice and dark colored urine due to the presence of hemoglobin (hemaglobinuria). When restricted to the morning hemaglobinuria may suggest paroxysmal nocturnal haemoglobinuria . Direct examination of blood under a microscope in a peripheral blood smear may demonstrate red blood cell fragments called schistocytes , red blood cells that look like spheres ( spherocytes ), and/or red blood cells missing small pieces ( bite cells ). An increased number of newly made red blood cells ( reticulocytes ) may also be a sign of bone marrow compensation for anemia. Laboratory studies commonly used to investigate hemolytic anemia include blood tests for breakdown products of red blood cells, bilirubin and lactate dehydrogenase , a test for the free hemoglobin binding protein haptoglobin , and the direct Coombs test to evaluate antibody binding to red blood cells suggesting autoimmune hemolytic anemia .
Plasma exchange therapy in autoimmune hemolytic anemia (AIHA) was used in four patients (two with warm hemolytic anemia and two with cold hemolytic anemia). The size of each plasma exchange approximated 1 plasma volume; three consecutive daily exchanges removed 80-90% of the immunoglobulins--immunoglobulin G (IgG) and immunoglobulin M (IgM)-, complement (C3, C4), and reduced antibody titers. Transfusion requirements dramatically decreased after plasma exchange in each case. In two patients, red blood cell (RBC) survival studies were performed to more accurately assess the effect of plasma exchange therapy, since steroid and/or immunosuppressive therapy was given concomitantly. In one case of cold AIHA, homologous 51Cr-RBC were injected 4 days prior to plasma exchange and repeat injection (same donor) following completion of plasma exchange. The survival curve prior to plasma exchange therapy had a T 1/2 = days (r = -) and after plasma exchange therapy had a T 1/2 = days (r = ). RBC survival studies using homologous 51Cr-RBC were also performed in a patient with warm AIHA. The survival curve before plasma exchange had a T 1/2 = 2 days (r = -), and postplasma exchange a T 1/2 = days (r = -). Plasma exchange therapy seems to have a beneficial effect in cold rather than warm autoimmune hemolytic anemia.