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How can autoimmune haemolytic anaemia be treated?

Q: I am a 42 years old man who has been suffering from autoimmune haemolytic anaemia for the past 5 years. I had 5 to 6 relapses even after taking Prednisolone and Azathioprene along with folic acid and calcium. Whenever I got a relapse I was asked to increase the Prednisolone dosage and later tapered it off. The lowest dosage that I could reach a year back was 7.5 mg / day. This year even with 10 mg/day of Prednisolone and 75 mg/day of Azathioprene I had 2 relapses. Is splenectomy the only option left for me? If so, is there any test to confirm the role of spleen in my case so that the success rate of the operation can be assessed?

A:Autoimmune haemolytic anaemia (AIHA) is a type of haemolytic anaemia where the body’s immune system attacks its own red blood cells (RBCs), leading to their destruction. Antibodies and associated complement system components become fixed onto the RBC surface. This leads to haemolysis, which can be intravascular (Red blood cell lysis occurs in the circulation as a result of activation of the complement system cascade) or extravascular (Red Blood Cells that are coated with antibodies are specifically recognized in the reticuloendothelial system and destroyed by macrophages). The spleen removes abnormal red cells from the circulation, including those labelled with antibodies. Removing the spleen can preserve those cells and prevent anaemia. Selection criteria for splenectomy for potential responders can be done using 51Cr-RBC sequestration studies (done at AIIMS haematology). However, there is a general criticism of such studies sine the results have been disappointing. Splenectomy may be the first choice of treatment in some types of haemolytic anaemia such as hereditary spherocytosis. However, in cases of autoimmune haemolytic anaemia, it is recommended when other measures have failed. Post splenectomy patients are prone to infections by encapsulated organisms. Immunization against infections with encapsulated organisms, such as Haemophilus influenzae and Streptococcus pneumoniae, as far in advance of the procedure as possible helps avert these problems. An appropriate haematology consultation will definitely help you select appropriate diagnostic approaches and laboratory tests and assist in planning and monitoring therapy.

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