Chronic Myeloid Leukemia | CML Specialist in Delhi | क्रोनिक माइलॉयड लुकेमिआ का इलाज
Chronic Myeloid Leukemia | CML specialist in Delhi NCR. Access best scientific, world-class treatment of CML in Delhi, Gurgaon, Faridabad, Noida. Formerly at AIIMS New Delhi for more than 12 years. Gold Medallist from AIIMS New Delhi.
What is Chronic Myeloid Leukemia (CML)?
Chronic Myeloid leukemia is a type of cancer of blood cells. It is one of the more common blood cancers among adults. Cancer can be defined as a condition where normal cells become abnormal and start dividing uncontrollably and spread in the body.
What is the difference between “chronic” (CML) and “acute” myeloid leukemia (AML)?
Acute myeloid leukemia (AML) is a completely different disease from CML. While CML is a slow-growing disease in which patients can live many years even without treatment, AML is a highly aggressive disease which results in death within a few months without treatment. Both CML and AML can be treated but the exact treatment differs.
Why does CML occur?
Normally, blood cells are produced from stem cells present in bone marrow. These are cells that divide and produce all other cells which are normally present in blood. In CML, a genetic abnormality (BCR-ABL, see below) occurs in some blood cells which leads to uncontrolled dysregulated division of cells.
No preventable “cause” of CML has been discovered so far except for exposure to radiation.
What are some common symptoms of CML?
The majority of patients with CML are asymptomatic, meaning that they have no symptoms. CML is usually discovered by an abnormal count noted in blood testing done for some other reason.
Occasionally patients with CML may have symptoms such as:
- Fever, tiredness, poor appetite and weight loss
- Enlargement of liver and spleen which is felt in the abdomen as a mass or heaviness
Uncommonly, patients may present in blast crisis (see below). Symptoms during blast crisis are usually same as those seen in acute leukemia with severe infections, bleeding, anemia, neurological symptoms, tumor masses etc.
Why is my doctor suspecting CML after looking at my blood counts?
Normally, total white blood cell counts (TLC) in adults are between 4000 and 11000 cells per uL. In CML, this count increases drastically (sometimes being in lakhs) with appearance of cells that are normally present only in bone marrow. This phenomenon is called “leukocytosis with left shift” and is typically seen in CML. If your doctor sees this kind of report, they will usually suspect CML.
What is a “leukemoid reaction” Is it cancer?
Leukemoid reaction is when the blood picture “looks like blood cancer but is not actually cancer”. It can happen due to a number of reasons, most importantly infection. An expert oncologist can help distinguish leukemoid reaction from leukemia.
Can CML spread from person to person?
CML is not a contagious disease and cannot spread from person to person.
How is CML diagnosed?
CML is characterized by a single, known genetic abnormality. This is called Philadelphia chromosome or BCR-ABL (see below). Your doctor will test for these by doing a blood or bone marrow study to confirm the diagnosis of CML.
What tests are required in a patient diagnosed with CML?
The following tests are required in patients of CML:
- Blood tests
- Bone marrow aspiration and biopsy
- Bone marrow cytogenetics for philadelphia chromosome
- BCR-ABL PCR testing in peripheral blood
- Additional testing in selected cases
What is bone marrow aspiration and biopsy?
Bone marrow aspiration/biopsy is a small procedure carried out under local anaesthesia. In it, a small needle is inserted into the pelvic bone and bone marrow is taken out for testing. The entire procedure usually does not take more than 15-30 minutes, and is generally done on outpatient basis.
In expert hands, bone marrow biopsy produces only minimal pain which recovers very soon.
Generally, bone marrow biopsy is a mandatory part of CML evaluation and must be done at least once for all patients.
What is meant by “cytogenetics” and “Philadelphia chromosome”?\
CML is characterized by a relatively simple genetic abnormality (compared to other cancers). This is called the Philadelphia chromosome. Two chromosomes (carriers of genetic information in cells) exchange an arm leading to formation of an abnormal fused structure which produces all the abnormalities seen in CML.
The Philadelphia chromosome can be seen in a special test performed on bone marrow. This is called “cytogenetics” or “karyotyping” and is a mandatory part of CML evaluation and follow-up.
What is meant by “BCR-ABL” and “PCR”? What is the difference between quantitative and qualitative PCR?
The Philadelphia chromosome translocation produces a fused gene which is called BCR-ABL. This gene can be detected in blood by a sophisticated technique called PCR (Polymerase Chain Reaction). So, PCR is the technology which allows us to detect BCR-ABL.
Qualitative PCR only detects if BCR-ABL is present or not. Thus, it is used to diagnose CML and not for follow-up.
Quantitative PCR tells us exactly how much BCR-ABL is present in blood, and it is used to monitor treatment and follow-up.
What are the stages of CML?
CML is divided into three main stages. The majority of patients are detected in chronic phase. This is called CML-CP.
If CML is kept uncontrolled for a long time, it may develop into an advanced stage. These stages are called accelerated phase (CML-AP) and blast crisis (CML-BC). They are discussed in more detail below.
How is CML-CP treated? Is chemotherapy needed?
CML chronic phase (CML-CP) is generally treated with a class of oral drugs called targeted therapy/tyrosine kinase inhibitors (TKI). These are drugs that specifically target the genetic mutation of CML and inhibit it, resulting in disease control with negligible side-effects. Chemotherapy is usually not required for treating CML-CP.
What is imatinib mesylate/Gleevec? How should it be taken and what are some common side effects?
Imatinib is the most common drug used to treat CML. As discussed above, imatinib is a targeted therapy and not chemotherapy. It falls in the class of drugs called tyrosine kinase inhibitors (TKIs).
Imatinib should be taken once daily, in the dose prescribed by your doctor, usually with food. It should be taken every single day and missed doses should be minimized as far as possible as it may lead to poor control of CML. If a dose is missed, no double dose is needed the next day but you should note it down and tell your doctor at the next follow up.
Imatinib is generally very safe and causes very few side effects. Serious side effects are uncommon. Many of these side-effects occur when the drug is first started and subside over time. Some side-effects that may be noted in a few patients include:
- Tiredness/fatigue or body aches
- Low blood counts
- Liver function abnormalities
- Fluid retention/edema
Can TKIs cure CML permanently?
Conventionally, it was believed that TKIs cannot cure CML. However, in recent years, it has been found that a few patients who have long durations of well-controlled CML may be able to stop taking the drug without relapse of disease. This is called treatment-free remission and is an area of active research. Approximately a third of patients who have well-controlled CML for many years may be able to quit drugs. However, they still need monitoring for relapse.
What is dasatinib (Sprycel) and nilotinib (Tasigna)?
Dasatinib and Nilotinib are two new drugs used to treat CML and are called “second-generation TKIs”. A small percentage of patients with CML-CP fail to respond to imatinib, or lose their response to imatinib after some years. Often, these patients can be treated successfully with these drugs. They are more potent and active than imatinib, but are also more expensive and have more side-effects.
Are there other TKIs that can be used in patients who cannot receive imatinib, dasatinib or nilotinib?
A few new drugs like bosutinib and ponatinib exist and can be attempted in these patients.
What is “IRMA”?
IRMA stands for “imatinib resistance mutation analysis”. In patients who fail treatment with imatinib, the choice of which TKI to use next can be clarified by IRMA testing. Some mutations are more sensitive to one TKI and others are more senstive to another TKI. Thus, IRMA helps us pick appropriate treatment in case of imatinib failure.
What is the best way to treat CML-CP if TKIs fail in my patient?
Some patients fail to respond to any TKI and suffer from persistantly active disease. Other patients may not tolerate any TKI due to side-effects. Such patients should ideally go for a bone marrow/stem cell transplant if possible. This option is discussed below.
How is accelerated phase CML (CML-AP) and blast crisis CML (CML-BC) treated?
Blast crisis (CML-BC) is generally very difficult to treat. Patients usually require some combination of second/third generation TKIs, intensive chemotherapy and bone marrow transplantation in an attempt to cure them.
Treatment of Accelerated phase (CML-AP) lies somewhere between CML-BC and CML-CP. Some patients may respond very well to TKI alone, while others may need more intensive therapy including bone marrow transplantation.
What is allogenic bone marrow transplantation/stem cell transplantation? When is it needed?
Allogenic Bone marrow transplantation/stem cell transplantation (BMT) is a procedure that involves:
- Eliminating the patient’s own hematopoietic (blood producing) and immune (infection fighting) system
- Replacing it with someone else’s (allogenic) system
In CML, bone marrow transplantation can produce permanent cure at all stages. However, allogenic BMT has severe side-effects in a significant minority of patients and can even cause death in some cases. Therefore, BMT is generally not used upfront in the vast majority of patients. BMT in CML is generally used in two scenarios:
- If TKIs are not working or not tolerated
- Advanced phase CML (CML-BC or CML-AP)
BMT is not advisable or possible routinely in every patient. Only relatively fit patients can tolerate BMT.
What is the long-term prognosis of CML?
The long-term prognosis of CML is excellent and the vast majority of patients will live their normal life-span with CML.
Why is it important to consult a CML specialist in Delhi?
The treatment of CML is showing evolution over the last few years. Several new drugs have appeared and the optimized sequencing of these drugs needs expert opinion. Molecular monitoring of CML is now routine and the possibility of treatment-free remission allows patients better, healthier lives. An expert is needed to navigate this complexity.
Dr Vineet Govinda Gupta is a medical oncologist based in Delhi NCR with expertise in the treatment of lymphoma. He has received his entire training from the apex medical institute of the country (AIIMS New Delhi) and been there for more than 12 years. He has received numerous awards including AIIMS Gold Medal for best Medical Oncologist and published research in cutting-edge journals. He also has significant experience in bone marrow/stem cell transplantation. More information here
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