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AIHA

 

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SUSAN LECLAIR

As I suspect you know, AIHA stands for auto-immune hemolytic anemia, a state that is diagnosed by the presence of antibodies on the red cells. The test is called either anti-human globulin test or the Coomb test. So - you already do have AIHA, most probably in the mild form called "compensated hemolysis". In compensated hemolysis, red cells are removed from the circulation earlier than normal but the bone marrow is capable of increasing its output to balance that early loss (compensation). A good indicator of the stress on the marrow in this situation is the reticulocyte count. This could be reported as a percentage or as an absolute number. What you shouuld see is increased numbers. The two issues here are 1)left to its own devices, the AIHA will increase and become uncompensated anemia which is harder to treat and will have all of the done side of anemia (fatigue, shortness of breath, pallor, lowered oxygen levels, etc. and 2) since your marrow is already stressed by the CLL, additional stress may have additional complications for the normal marrow cells.

One very common treatment for AIHA is prednisone of some dosage. The idea is to lessen the cells's ability to make antibodies which cause the AIHA. Along with the usual down side for prednisone use, the idea that you want to lessen antibodies makes one more at risk for infections, etc. There are others - going all the way to a splenectomy which doesn't do a thing to the production of the antibodies but does take away the site which removes the antibody coated red cells from the circulation.

SUSAN LECLAIR

My guess is that the reason there is so little out there about AIHA is that it is not a new disease so most medical types already know what they need to know about it.

In a nutshell - lymphocytes - B lymphocytes to be precise - make antibodies. The antibodies are to be made in response to the presence of foreign proteins and other antigens. The antibodies are to be make as long as it takes to rid the body of the foreign antigen. After the elimination is complete, the cells responsible for the antibody either die (the majority) or they get modified into memory cells which will remember the antigen and how to make the antibody, thus giving the person protection against the antigen (how vaccinations work).

CLL is most often a disease of B cells. So it is possible - sometimes even common - for these malignant B cells to make abnormal antibodies which attack the body itself. One of the most common of these mis-directed antibodies is the red cell. So you have a situation in which your own immune system is destroying your own red cells (auto-immune hemolysis).

Usually the approach is to prevent the making of the antibody which is done through the use of everyone favorite (not) drug - prednisone. Prednisone suppresses the B lymphocyte's ability to make antibodies. One of the problems is that it will suppress all B cells, not just the malignant ones so you are at risk for not responding appropriately when confronting a new antigen. Prednisone also has some other side effects which are not all that pleasant - weight gain, mood swings, etc. After 1-2 courses, the cells usually will stop making the offending antibody. Some people think that they "forget" rather easily. Sometimes the AIHA comes and goes with changes in the disease progression.

If, after multiple attempts to fix this problem, it still won't go away, the last recourse is a splenectomy. Why? Because antibody coated red cells are hemolyzed in the spleen. If you take out the spleen, then there is no place for these coated cells to be killed and they stay around longer. The interesting thing is that these red cells function quite well even when there is an antibody attached to them.

TERRY HAMBLIN
Recent results suggest that Fludarabine plus cyclophosphamide is less
likely to trigger AIHA than fludarabine alone.
TERRY HAMBLIN

May 2005

Although treatment contributes to both hypogammaglobulinemia and to AIHA, they are intrinsic properties of CLL, and may occur without treatment.

Some cases of CLL present with AIHA, and indeed once the AIHA has been treated with steroids, the CLL is barely detectable. I would say that at least one third of cases in my experience occur without the patients having been treated.

Some degree of hypogammaglobulinemia occurs in about 60% of CLL patients and it is progressive. The longer you have CLL, even the w & w variety, the more likely you are to get hypogammaglobulinemia.

There are lots of theories why patients get these complications, but no-one yet ha a perfect answer.

Incidentally, it is not necessary to go to hospital to have iv Ig. In Sweden the rule is for patients to self-inject and have the Ig as a sub-Q infusion at home.

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