Autoimmune hemolytic anemia basics – pro

Lay Summary: I discuss some very basic facts about AIHA.

Autoimmune hemolytic anemia (AIHA) due to the presence of warm agglutinins is almost always due to IgG antibodies that react with protein antigens on the red blood cell (RBC) surface at body temperature. For this reason, they are called “warm agglutinins” even though they seldom directly agglutinate the RBCs. IV Gammaglobulin blocks this process.

I some cases, AIHA can be characterised by a chronic course and an unsatisfactory control of haemolysis, thus requiring prolonged immunosuppressive therapy. Sometimes when medical measures fail, it may be necessary to surgically remove the spleen (splenectomy). The clinical course of the disease may show either resistance to steroids or dependence on high-dose steroids with subsequent development of severe side effects on growth, bone mineralisation, and the endocrine system. Splenectomy is effective in about 50 to 60 percent of the time in IgG antibody diseases but is not usually effective in IgM antibody haemolysis. Splenectomy is of benefit in these people because the spleen behaves like a sieve and if it is removed, even though the RBCs are coated by antibodies, they are no longer caught and destroyed in the spleen.

IVIG is an accepted treatment for autoimmune hemolytic anemia. Unlike steroids, it does not induce remissions but is a temporizing measure until a definitve treatment can be planned and delivered.  IVIG is not as effective in AIHA as it is in ITP. Other treatments can sometimes be used.

Autoimmune hemolytic anemia can be associated with lymphoproliferative conditions and lymphoma.  A clinical examination (to rule out lymphadenopathy, splenomegaly) is obligatory. The need for additional investigations must be determined by history, clinical findings, and the type of antibody. Routine work-up relevant for treatment decisions may include abdominal examination by computed tomographic scan (to search for splenomegaly, abdominal lymphomas, ovarian dermoid cysts, renal cell carcinoma), quantitative determination of immunoglobulins, a search for a lupus anticoagulant in case of warm antibodies, or a bone marrow examination and a search for clonal immunoglobulins (immune fixation) in case of cold antibodies.

Ucar K. Clinical presentation and management of hemolytic anemias. Oncology [Huntingt] 2002;16(9 suppl 10):163-70.

Schwartz RS, Berkman EM, Silberstein LE. Autoimmune hemolytic anemias. In: Hoffman R, Benz EJ Jr, Shattil SJ, Furie B, Cohen HJ, Silberstein LE, et al., eds. Hematology: basic principles and practice. 3d ed. Philadelphia: Churchill Livingstone, 2000:624.

Klaus Lechner and Ulrich Jäger, How I treat autoimmune hemolytic anemias in adults. September 16, 2010; Blood: 116 (11)

So Yeon Park et al, A Case of Non-Hodgkin’s Lymphoma in Patient with Coombs’ Negative Hemolytic Anemia and Idiopathic Thrombocytopenic Purpura. Cancer Res Treat. 2012 Mar; 44(1): 69–72.

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