Autoimmune hemolytic anemia is an "Abnormal immune function in which a person's immune reactive cells fail to recognize his or her own red blood cells as self cells." (Byar, 2010, pg. 893) Cold Autoimmune Hemolytic Anemia also known as CAHA was first discovered by Landsteiner in 1903. "He described the finding of a low titer of these agglutinins in healthy individuals. Later, the appearance of the I antigen on human RBCs in the postnatal period due to modification of the fetal I antigen structure (a change occurring over the first 18 months) was found to lead to the development of low levels of anti-I agglutinins. These antibodies induce hemagglutination mainly at 4 degrees C but not at 37 degrees C and were therefore termed cold agglutinins."(Georgy 2010) The actual pathology in which the action to the red blood cells and the vessels occurs was first determined years later by Clough and Iwai. Then in 1953, Schubothe named it Cold Agglutinin Disease, or CAD. Cold autoimmune hemolytic anemia, also known as cold agglutinins, is a rare anemia that produces immunoglobulin M which attacks the I/i and H antigens of the red blood cells. This causes an agglutinin affect, sticking or clumping of the red blood cells and the patient begin to suffer from peripheral "numbness" and "bluing" do to the lack of circulation. The small vessels in combination with the agglutinin do not allow circulation of the blood through those areas.
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Cold autoimmune hemolytic anemia is a rare anemia that mostly affects the elderly population, though it can affect anyone at any age. When it happens to the elderly, it is usually due to lymphoproliferative disorder. When it affects children it is usually due to an infection such as mycoplasma pneumonia, infectious mononucleosis, varicella, rubella, and parvovirus B19. CAHA affects about one in eighty thousand people in the population. Females are more prevalent with a ratio of 2 to 3 more likely than males. Death due to CAHA is rare and is almost nonexistent.
There are three types of autoimmune hemolytic anemia, warm, cold and medicinal drug related. There are many diseases that stem from this anemia. One disease that is associated with cold hemolytic anemia is Raynaud's Disease. This is the irregular incidents of arterial spasms of the ears, nose, toes and fingers. This disease happens mostly to females between the ages of fifteen and forty five. There is warm and cold anemia due to the way they react to different temperatures. In a patient with CAHA, the patient is producing immunoglobunin antibodies against their own red blood cells.
In the warm autoimmune hemolytic anemia the cause is most often not known. This anemia is most common among females in the population. The usual symptoms are jaundice, paleness and splenomegaly. It is linked to rheumatoid arthritis, chronic lymphocytic leukemia, and myeloma. In the warm autoimmune hemolytic anemia, the antibodies in the body attack and merge with the red blood cells at normal body temperatures.
The drug induced hemolytic anemia causes about twenty percent of the cases. The drugs that are mostly associated with this are methyldopa, penicillin, quinine, and quinidine. This is caused by the body creating irregular amounts of antibodies in response to the drug.
There are two kinds of cold autoimmune hemolytic anemia. They are primary and secondary. There are no known causes for the primary form but the secondary one is caused by infections or lymphoproliferation. "In most cases, CAHA is a primary disorder that typically becomes apparent at 50 to 60 years of age. Symptoms and findings associated with the disorder may include fatigue; low levels of circulating red blood cells(anemia); persistent yellowing of the skin, mucous membranes, and white of the eyes(jaundice); and/or sweating and coldness of the fingers and/or toes(digits) and uneven bluish or reddish discoloration of the skin of the digits, ankles, and wrists(acrocyanosis or Raynaud's sign).cold antibody hemolytic anemia may also occur as secondary disorder in association with a number of different underlying disorder such as certain infection diseases(e.g., mycoplasma infection, mumps, cytomegalovirus, infectious mononucleosis), immunoproliferative diseases(e.g. non-Hodgkin's lymphoma, chronic lymphocytic leukemia), or connective tissue disorder(e.g. systemic lupus erythematosus). Although CAHA is known to be an autoimmune disorder, its exact underlying cause is not fully understood."(WebMD 2010) When the CAHA is acquired through an infection, the disorder usually disappears within six months but if it is caused by lymphoproliferation then it becomes a chronic condition in which there is no real cure.
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Cold autoimmune hemolytic anemia antibodies are different than the warm autoimmune hemolytic anemia because agglutinin occurs at a lower temperature. The binding occurs at 28 to 31 degrees C. The activation of the antigens usually occurs at twenty to twenty five degrees Celsius but it is also possible at normal body temperatures. The cold agglutinin antibodies attack the red blood cells. Anemia occurs in the body when hemoglobin is released due to red blood cell obliteration. The red blood cells are destroyed faster than the bone marrow can produce.
Since the CAHA bind better in cold temperatures, it affects the more distal parts of the body. It affects those body parts due to their small vessels and the propensity for those body parts to be colder than the rest of the body. When the agglutinates bind the blood in those parts of the body, the vessels are so small that it causes the blood to clog up and lose circulation. This is what is happening to the body parts become numb or start to turn blue.
The titer of the antibodies reaches its maximum usually at about two to three weeks after the onset of the infection and show on the tests for up to two to three months.
ANALYSIS OF CLINICAL FINDINGS/ MANIFESTATIONS
The patient is sixty two year old Hispanic female. She has diagnosed with cold agglutinin antibody hemolytic anemia and has been admitted thirteen days ago for acute decompensated heart failure with symptomatic anemia. Her decompensating heart failure is due to her diastolic dysfunction and her anemia. She is dependent on transfusions due to her anemia.
At admission she seemed dehydrated and malnourished. She was pale in color, and tachycardiac with a heart rate of 98 at rest. Her blood pressure was at eighty four over forty seven mmHg. The assessment of the lungs showed crackles to the mid lung fields bilaterally. Her O2 Sat was at 97 percent with a capillary refill of greater than eight seconds. She has had blood drawn for labs antibody titers.
She has the signs of cold autoimmune hemolytic anemia. She is pale in color, and complained of tingling in her fingers. At this point she has a capillary refill greater than eight seconds. Just on visual sight, it is noticeable that her fingers are turning cyanotic. Because her condition of CAHA had already been diagnosed in her previous visits the search of her symptoms was much easier and determining the course of action is clearer.
All the signs and symptoms correspond to what is written in the literature. She is a female which consists of most of the cases of CAHA. She matches the age group that is most likely to be affected. But her basic signs of pallor, tachycardia at rest and fatigue are a regular symptom.
ANALYSIS OF LABS/DIAGNOSTIC TESTS
The Coombs' test is performed on patients that are suspected of having autoimmune hemolytic anemia. There are two forms of the test, direct and indirect tests. The direct Coombs' test checks for antibodies that have that have already bonded to the red blood cells. This test is also used to determine anemia or the possibility of jaundice. The indirect Coombs' test searches for the antibodies that have not bound to the red blood cells. This test is not used to determine a disease but rather establish whether or not the person would have reaction to a blood transfusion.
A normal or negative result would indicate that no antibodies were found. There are no antibodies attached to the red blood cells in a direct test. In an indirect test, this would mean that the patient has a blood that is compatible with the donor's blood.
An abnormal or positive test result for a direct test would indicate that the antibodies are binding to the red blood cells and attacking them. This would indicate a possibility of anemia. There are other factors that have to be taken into consideration when there is a positive result. One of the considerations may be the fact that the patient has received incompatible blood. In the indirect test, a positive result would indicate that the patient's blood is not compatible with the donor's blood.
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There are a few things that may affect or skew the test results. If the patient has had a blood transfusion in the past, this may give a false positive. The false positive may be due to the fact that patient received the wrong blood. If the patient was pregnant in the last three months, a Rhesus factor may occur. This is when a mother may have an Rh positive blood and the baby has an Rh negative blood or vice versa. When this occurs, some of the baby's blood may get mixed in with the mother's blood causing her antibodies to attack the baby's blood. During the Coombs' test, this would show up as positive for agglutinin. And there are some medications that may affect the results. They are such medicines as cephalosporins, sulfa medicines, tuberculosis medicines, insulin and tetracyclines.
The lab results showed a Hemoglobin 8.2 which is low with the normal range being 12- 16G/dL. The Hematocrit was 24.5 which are low with the normal range being 36- 46 percent. The hemoglobin that is low is an example that she is showing signs of anemia.
Here is an example of steps taken when the lab receives the blood for analyses;
"1) Complate blood cell (CBC) including the reticulocyte count, the RBC count and Hematocrit are decreased, the MCV falsely elevated and the MCH and MCHC are falsely decreased. The reticulocyte number may be increased depending on the status of the disease and its severity. Depending on the severity of the disease, many erythrocyte aggregates are found. The monospecific direct antiglobulin test (DAT) is positive (complement C3D-specifity).
2) The titer of anti-I is increased.
3) More nonspecifically increased analytes are: bilirubin, LDH, AST, serum- hemoglobin.
4) Haptoglobin is decreased.
5) Bone marrow examination is necessary for the evaluation of the erythropoiesis and to search for possible causes of the disease (e.g., lymphoma). In most cases erythropoiesis is increased to compensate for vigorous hemolysis.
6) Microbiological and virological diagnostics (possible Mycoplasma or EBV infection) Mycoplasma pneumonia infection is sometimes accompanied by a marked increase in anti-I titer."(Stamminger and Beier 2007, pg3-4)
The patient was given a transfusion of red blood cells, and Lasix was also given. The transfusion was given to replace the destroyed red blood cells in her system. The Lasix was given to remove excess fluid in her body. The transfusion led to her fluid overload so Lasix was given. At this time this seemed to the best course of action to take to alleviate her anemia. The patient was kept warm to prevent agglutinin of her blood. This corresponds to the treatment course that is stated in most of the literatures. The Medical Surgical Nursing is also congruent with most of the literature by stating that the course of action should be immunosuppressants first then plasma exchange when medication does not respond. (Byar 2010, pg899)
The treatment of cold autoimmune hemolytic anemia differ according to the patients needs. The treatments include blood transfusions, medicines, plasmapheresis, surgery, marrow and blood stem cell transplant, and a change lifestyle. The treatments are given on the mildness or severity of the condition. A mild case of CAHA may not need treatment but if the anemia is severe than the patient may need pursue ongoing treatments. The basic goal for treating CAHA is to stop or at least reduce the destruction of the red blood cells and bringing back up the levels of RBC in that patient. The second goal would be to treat the actual cause of the anemia.
The use of blood transfusion is for a severe or life threatening situation. Doing a blood transfusion is a little more extensive since the blood needs to be type and crossed and has to be given intravenously. The matching of the blood is a crucial part of this procedure to ensure the survival of the patient.
There are medicines that can improve certain types of autoimmune hemolytic anemia. Corticosteroid medications are used especially prednisone. This is normally used for warm autoimmune hemolytic anemia. Prednisone is an immunosuppressant drug so this leaves the patient with a risk obtaining other infections but with careful planning, the patient will do well. This suppresses the immune system in the body so that the body is prevented from making antibodies which attack the red blood cells. For CAHA, the most successful drug to use has been rituximab. "Berentsen performed an open, uncontrolled prospective phase two study of rituximab in CAD. Twenty of twenty seven patients responded, but almost all responses (n = 19) were PRs. The median duration of response was 11 months, and most of the relapsed patients responded to retreatment with rituximab. Similar results were obtained by Schollkopf. We treat all our patients with symptomatic CAD (hemoglobin level below 9- 10 g/dL and/or vascular symptoms) with rituximab. Remarkable responses have recently been obtained with eculizumab and bortezomib in rituximab-refractory patients." (Lechner and Jager, 2010, pg1836)
Plasmapheresis is also a form of treatment that deals with the immune system. During this procedure, the antibodies are removed from the blood. This procedure consists of removing the blood from the body and separating the blood from the plasma. Then donor plasma is combined with the patient's blood and is placed back into the patient through an I.V. There are some people who may require having surgery. There are two procedures that can be performed. One is the removal of the spleen and the other is the blood and marrow transplant. With every surgery there are risks and it may not be a cure for the condition.
One of the procedures is the removal of the spleen, Splenectomy. This is performed most often in warm autoimmune hemolytic anemia patients and rarely is associated with CAHA patients. The spleen is the organ that fights infections and removes damaged blood. But when the spleen is diseased, it takes out more red blood cells than necessary and for an anemic patient this becomes a problem. So removing the spleen would reduce the amount of red blood cells being filtered out. Also a patient who has had a Splenectomy has reduced immune function and is also unable to rid themselves of disease causing organism and are at a greater risk for infection and sepsis. (Workman 2010, pg879) The consequences of this surgery have great implication on the patient so many times this is the last resort.
In some hemolytic anemia, the marrow does not make the necessary red blood cells for the body. Then sometimes the red blood cells made in the marrow are destroyed before it reaches its maturity. The transplant is necessary to replace the damaged marrow. The difficulty or drawback to a transplant is locating a match donor. During this procedure a blood transfusion is done with the stem cells placed in them. "The goal is for the blood to carry the stem cell to the marrow and start making new healthy blood cells."(Diseases and Condition 2010)
The easiest of all treatments may be just a change in lifestyle. When a person has a mild case of CAHA, avoiding cold weather or exposure to cold may be the easiest resolution. Some examples may be; "wear gloves or mittens when taking food out of the refrigerator or freezer. Wear a hat, scarf, and a coat with snug cuffs during cold weather. Turn down air conditioning or dress warmly while in an air-conditioned space. And warm up the car before driving in cold weather.
The most common treatment for CAHA is the change in lifestyle. The doctors and the nurses will coordinate with the patient as to a program in which he or she can follow to reduce or prevent future occurrences. They would inform the patient to avoid exposure to the cold and to be aware of their bodies. They need to be vigilant in tracking how their body is reacting to the cold.
Other than her heart condition, this patient should be able to function normally. She would have to ensure that she remains warm at all times and be aware when she is going out or sleeping with the possibility that it may become cold. Her survival rate and longevity of life is only determined by her other diseases and there severity. As long as she is able to correct or control her other health issues, Cold Autoimmune Hemolytic Anemia should not factor into her daily activities. She can go back to doing what she did in the past. She can maintain her activity level and live a long life. Her only financial cost or affect would be that she would need to buy clothes that allowed her to be in colder areas. She would also have to supplement dietary intake of iron to support her red blood cells. So as a patient, her outcome after CAHA is a positive one and hopefully she will able to live out a fruitful life.