I am not sure how many of you got this, but if you guessed hemolytic anemia YOU ARE RIGHT!!! BUT, did you guess cold autoimmune hemolytic anemia? Well, if you were a REAL doctor, instead of playing doctor, you would have been asking yourself, is this warm or cold hemolytic anemia. And if you were a real doctor, you knew you would have to wait for the lab results to confirm. So here are results from some of the lab tests the real doctors ordered: • DAT (direct antiglobulin test) has come back positive, which is a test used to find out whether a hemolytic anemia is immune-related or not. A positive DAT confirms Wayne’s hemolytic anemia is a result of an auto-immune problem. • With Antisera reacting to complement –positive for M this is a matter of complement, a protein that binds to the red cells. Having this is not good, because complement pokes holes in red cells! That’s where the hemolytic part comes in with cold AIHA – the red cells get busted open by complement, right in the circulation. (FOR all you out there who can speak Medical talk – Cold agglutinin disease is typically characterized by the presence of IgM antibodies directed against polysaccharide antigens on the red blood cell surface. Right …?)
Immune hemolytic anemias referred as autoimmune hemolytic anemias (AIHA), come in two kinds: warm and cold. They are named that way because the different antibodies in each type react best at different temperatures. There are a couple things going on in cold autoimmune hemolytic anemia (AIHA): 1) the patient is making antibodies against his/her red cells, and 2) there is complement fixed to the patient’s red cells. The possible causes are numerous and include infections, blood disorders and the good old “idiopathic” category (or, We Don’t Know What Is Causing It But We Don’t Want To Sound Silly). In Wayne’s case, you can add to the list of culprits cGVHD (chronic graft vs host disease).
Wayne is making IgM antibodies. These antibodies bind best at cold temperatures. So, did he get really cold? NO. Oh I hear many out there saying, “it’s not surprising Wayne developed cold AIHA, Peeches would never turn on the heat.” If you could see him now, it would be hard to believe that Wayne could be producing any IgM antibodies, the way he likes to bundle up. Hmm…Wayne has always had trouble with cold hands, I wonder if that was a predisposition to developing cold AIHA, (a thought of mine from left field)???
So what are they going to do!!! They are giving him a special medicine that will bind to the antibodies, Rituximab. Rituximab targets a protein on these cells, which are part of the immune system (the body’s defense against infections and other harmful substances) by temporarily removing the “arrant” cells. This takes about 6 weeks, so until that time he will continue to receive transfusions every couple of days. BUT…..drum roll……. he doesn’t need to stay in the hospital for all that time, so maybe if the stars align we will be home before.