Hemolytic anemia autoimmune type

The effectiveness of immunosuppressive drugs in autoimmune disorders varies widely. Nonetheless, with immunosuppressive therapy, remissions can be obtained in many instances of autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, type 1 diabetes, Hashimoto s thyroiditis, and temporal arteritis. Improvement is also often seen in patients with systemic lupus erythematosus, acute glomerulonephritis, acquired factor VIII inhibitors (antibodies), rheumatoid arthritis, inflammatory myopathy, scleroderma, and certain other autoimmune states. 

Autoimmune diseases have been reported to be more frequent in human subjects treated with several recombinant cytokines [38], For instance, increased titers or the new occurrence of autoantibodies have been observed in hepatitis C patients treated with the recombinant interferons-alpha (IFNa). Quite a few clinical case reports describe the development of organ-specific as well as systemic autoimmune diseases including systemic lupus erythematosus, insulin-dependent type I diabetes mellitus, autoimmune thrombocytopenia, autoimmune hemolytic anemia, myasthenia gravis, and autoimmune thyroiditis in patients under IFNa therapy. Although the mechanism involved is not fully elucidated, the available data support the pathogenic potential of IFNa in autoimmunity [31]. In contrast, autoimmune effects associated with IFNp therapy are thought to be of lesser concern based on the current clinical evidence [38], Thyroid autoimmunity in contrast to other autoimmune diseases is frequent in patients treated with recombinant interleukin-2 (rIL-2). Thus, among 281 previously euthyroid cancer patients treated with rIL-2, up to 41%... 

In addition to cancer, too little apoptosis can also result in diseases such as autoimmune lymphoproliferative syndrome (ALPS). This occurs when there is insufficient apoptosis of auto-aggressive T cells, resulting in multiple autoimmune diseases. An overproliferation of B cells occurs as well, resulting in excess immunoglobulin production, leading to autoimmunity. Some of the common diseases of ALPS include hemolytic anemia, immune-mediated thrombocytopenia, and autoimmune neutropenia. The different types of this condition are caused by different mutations. Type 1A results from a mutation in the death domain of the Fas receptor, Type IB results from a mutation in Fas ligand, and Type 2 results from a mutation in caspase 10, reducing its activity. 

Type II, or cytolytic, reactions are mediated by both IgG and IgM antibodies and usually are attributed to their ability to activate the complement systerrr The major target tissues for cytolytic reactions are the cells in the circulatory system. Examples of type II allergic responses include penicillin-inhemolytic anemia, methyldopa-indMced autoimmune hemolytic anemia, quinidine-induced thrombocytopenic purpura, and sulfonamide-induced granulocytopenia. These autoimmune reactions to drugs usually subside within several months after removal of the offending agent. 

As already discussed in Sect. D.1.2, immunologically mediated hemolytic anemia may be due to three different mechanisms, defined as the hapten-cell binding type, the innocent bystander type, and the autoimmune type. The drugs most frequently responsible are given in Table 6. 

Some other drugs produce an autoimmune hemolytic anemia in which the antibodies are directed towards native antigens on the red cell surface, while the drug does not seem to function as a hapten in the reaction. This type of autoimmune hemolytic anemia has been described following treatment with levodopa, methyl-dopa, and mefenamic acid (Worrledge et al. 1966 Worrledge 1973). With a-methyldopa, clinically significant hemolysis develops in less than 1 % of individuals... 

L-Dopa produces some of the same manifestations that a-methyldopa produces, including sensitization of red cells by antibody (with or without hemolytic anemia), drug fever, rash, and leukopenia, but all of these manifestations are rare. The hemolytic anemia produced by L-dopa is very rare. It appears to be of the autoimmune type, involving Rh determinants analogous to the much more common hemolytic anemia seen with a-methyldopa therapy (Worlledge 1973). L-Dopa has also been reported to produce an acute non-hemolytic anemia (Alkalay and Zi-POLi 1977). 

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