CLL - FAQ

Following are some of the questions to ask in early visits to your hematologist:

What do the blood tests show? How is this different from "normal"?
What stage am I in?
Will I need further tests? How often will they be?
Will you mail or fax copies of my test results, or should I come in to pick them up? If I pick them up, when will they be ready?
What symptoms should I watch for?
What changes should I make to my daily routine to avoid complications?
When do I see you and when do I see my family Doctor?
When do you anticipate that I will require treatment?
How many CLL patients do you have?
How do you anticipate treating my CLL?
What is the long-term outlook for my CLL?

CD38 is an antigen that is often found on the surface of B-CLL cells. It is detected by flow cytometry, a diagnostic technique that is frequently used in confirming CLL. The presence of CD38 may enable physicians to predict with considerable accuracy whether a patient is likely to have a favorable or unfavorable clinical course.

Patients with less that 20 percent CD38+ B-CLL cells are likely to have a favorable clinical course requiring minimal or no therapy. Patients with equal to or greater than 20 percent CD38+ B-CLL cells are more likely to have an unfavorable clinical course requiring earlier and ongoing treatment. Significant differences in survival are also thought to exist between these two groups. CD38 expression is relatively stable over time and does not seem to be influenced by chemotherapy.

CD38 seems to be closely linked with Ig V gene mutation, and both are thought to be useful prognostic indicators in B-CLL. There is a strong correlation between CD38 expression and Ig V gene mutation-lower CD38 expression correlates with a higher incidence of Ig V gene mutation. However, because of the relative ease of testing for CD38 expression, it is a more convenient test.

CD38 is one of a number of prognostic indicators in CLL. Others include, circulating levels of beta-2-microglobulin and soluble CD23, lymphocyte doubling time, serum thymidine kinase levels, bone marrow histology, and chromosome abnormalities.

Flow cytometry is a diagnostic technique that is used to measure the chemical or physical characteristics of cells in suspension. Flow cytometry can also determine the types and the quantities of antigens expressed on cell membranes through a process called immunophenotyping. Antigens are substances that are capable of activating the immune system. Each antigen category is called a cluster of differentiation (CD) and is numbered.

This is very much an evolving field. Isolation of CD markers is continuing and new ones are being found monthly. As a result, marker panels will change, and more specific information will be available. As a general rule, it can be said that,

Cells that are positive for CD2, 3, 4, 5, 8, 45RA and/or 45RO are T lymphocytes.
Cells that are positive for CD10, 19, 20, 21, 23, 35, 40, and sometimes 77 are B lymphocytes (CD5 can also be found on a specific subset of B lymphocytes).
CD 28 can be positive with T or B cells and NK (natural killer) cells, which are another lymphocyte subset.
CD 34 is one of the most important markers for it is seen in the hematopoietic pluripotential stem cell (PSC) which is the cell that is "wanted" in bone marrow or peripheral blood stem cell transplants.
CD 38 is a receptor that is found in plasma cells, some thymocytes (early lymphocytes still in the thymus), NK cells, and in very early B cells. Increased presence of CD38 has been found in cells in multiple myeloma, and certain acute lymphoblastic and myeloblastic leukemias. CD38 is also thought to be of predictive value in determining the clinical course that will CLL will take.

In CLL cells, a distinct pattern of antigens is expressed. CLL lymphocytes coexpress the B-cell antigens CD19 and CD20 along with the T-cell antigen CD5. The analysis of a blood sample by flow cytometry is therefore very useful in confirming the diagnosis of CLL.

The following diagram illustrates how CD markers are used in the diagnosis of disorders that are characterized by elevated lymphocyte counts (lymphocytosis). It is presented here with permission of the copyright holder, Dr. Margaret Uthman.

The terms kappa and lambda which appear on flow cytometry reports refer to portions of the immunoglobulin or antibody molecule. Kappa and lambda are long chains of amino acids. While there are heavy chains and light chains, kappa and lambda are both light chains. Most people should be able to make either of these light chains in the amounts appropriate for antibody activity. Over expression of one of the chains is often seen in CLL patients, and usually means a loss of control by the cells.

When the leukemias were first discovered, the only tool available was the microscope. Now we have learned how to identify cells by their cell membranes. It is hoped that we will soon be able to identify cells by their DNA.

Flow cytometry is very time consuming to perform. Cells cannot be tested for every known antigen-it's too expensive, requires too much time, and there is too great a possibility of error. For these reasons, every lab makes a series of decisions concerning which tests will be run on which samples.

In CLL, malignant lymphocytes derive from cells gone berserk (monoclonal expansion). If we can find out what type of cell it is (for example, does it have a compound on its membrane that is called CD20), then it is possible to design a drug (monoclonal antibody) to fight only CD20+ cells. This is the underlying principle of medications like rituximab (Rituxan).

At this time there are no proven complementary therapies for use in the treatment of CLL, and patients are advised to avoid herbal supplements like Echinacea that stimulate the immune system.

If any reaction occurs during administration, the treatment is stopped and the patient may receive another dose of Tylenol, Benedryl, and/or Demerol.

In addition, it may be helpful to take two Tylenol PM (or generic) each night before the morning's treatment. This may help to avoid side-effects during treatment. A patient should also take a normal dose of Tylenol every 4 hours after treatment if he/she runs a fever after receiving Campath.

After the first week, Campath will be administered at 30 mg, with pre-treatment of Tylenol and Benedryl, typically on Monday, Wednesday, and Friday. If a holiday occurs on Monday and your doctor's office is closed, you may receive a dose on Tuesday, Wednesday, and Friday. If Campath treatments are stopped for ANY reason for 7 days or longer, the patient must start over with the dose escalation (3 mg, 10 mg, and then 30 mg).

If any side effects occur that concern the patient and/or staff, the infusion should be stopped. The patient should be hydrated with IV saline for 20 to 30 minutes before another try is made to infuse the Campath.

If a patient on Campath-1H develops ANY symptom of an oncoming infection (fever, cough, runny nose, sore throat, sores in mouth, stomach cramps or diarrhea, or an overly red and/or inflamed cut or scratch on the skin), the patient should contact the doctor IMMEDIATELY!