The Professors' Posts

Anemia

 

ACOR CLL List Help Pages
SUSAN LECLAIR

That there is a hemolytic anemia is usually diagnosed by an increased reticulocyte count and a decreased red cell lifespan. There are literally hundreds of different types of hemolytic anemia so we need to sort some of the mechanisms out. Typically, the category of hemolytic anemia is subdivided into two major branches - intracorpuscular (something is actually wrong with the red cell itself) and extracorpuscular (something from somewhere else is damaging the cells causing them to die earlier than they should.)

Intracorpuscular causes include: * Abnormal hemoglobins (the most well known one is sickle cell hemoglobin). There are over 600 different abnormal hemoglobins. The vast majority of these are inherited so since your father in law is 72 and then just happened to him, we can assume that these are not the problem. Regardless of how you get an abnormal hemoglobin, there are specific tests to identify them. * Abnormal enzyme activity. Although a much lower number than abnormal hemoglobins, most of these are inherited and similar to the hemoglobinopathies, there are specific tests for most of these as well. * Abnormal membrane function. If the abnormality in the membrane is the result of a problem in the construction of the membrane, then it is called an intracorpuscular defect. Again, my sense is that this is not his problem.

Extracorpuscular causes include: * Physical means include changes in temperature, radiation, etc but these are easily ruled out in the history or physical. There is a group of hemolytic anemias called microangiopathic in which damage to small blood vessels causes the cells to rupture. Examples include if there is excessive clotting or tumors or scarring. * Chemical means both external or internally produced compounds. So - if you decided to drink lysol, your cells would hemolyse due to an external chemical cause and if your kidneys did not work and the level of waste got to a certain level, that "poison" would destroy your own red cells. * Imunologic. You decide to make antibodies against your own cells (autoimmune hemolytic anemia or AIHA) or you have had a recent transfusion and the plasma which you received had pre-formed antibodies against some antigen on your cells or the red cells which you received have an antigen to which you already have an antibody. * Who knows. Yes, there are a lot of these. In these situations, every specific test for the cause of the hemolysis is negative yet hemolysis continues.

Your father in law's physicians will be doing a lot of screening tests to try and figure out what is happening.

Procrit is used to stimulate more cell production so it probably won't be used until they figure out what is causing the hemolysis. Otherwise you will simply be making more cells that will die early.

Terry Hamblin

19th June 2005

Anemia caused by IHA, marrow failure or MDS.

Immune hemolytic anemia (in this case the same as AIHA) is diagnosable by the presence of a positive direct Coombs test (or antiglobulin test). You would also expect to see reduced haptoglobins, increased urinary urobilinogen and increased reticulocytes.

MDS is a form of bone marrow failure. A blood film might be expected to show the characteristic features - irregularly shaped red cells, hypogranular neutrophils, uni- and bi-lobed meutrophils, giant agranular platelets, but it is best diagnosed by a bone marrow. Here the changes in the size and shapes of developing blood cells would be easier to recognise and in addition chromosomal analysis should be done since the features of secondary MDS are well recognised.

Another possible diagnosis is marrow infiltration with CLL, and this will onjly be diagnosed by a bone marrow biopsy.

Patients who have low levels of platelets, neutrophils and red cells after treatment for CLL are in a difficult diagnostic situation. It could be an immune effect, but this should be easy to diagnose. The alternative is that the treatment has damaged the bone marrow stem cells. This could either mean that few if any blood cells can be produced or it could mean that the cells that are produced cannot mature properly into full-grown blood cells. The latter is MDS and is recognised by what the cells look like down a microscope.

There is little that can be done in either situation except supportive care, which generally means blood transfusions, perhaps with platelet transfusions and antibiotics when necessary. New drugs for MDS such as 5-azacytidine and Revlimid have not been shown to be of value in secondary MDS.

Return to Professors' Posts

Help Page