Overview:
Chronic neutrophilic leukemia (CNL) is a cancer of the blood system. CNL is known as “myeloproliferative neoplasm,” in which the bone marrow produces abnormal blood cells. The cells so produced look and behave abnormally. CNL is a rare disorder globally; only 200 cases have been diagnosed so far. In chronic neutrophilic leukemia, neutrophils - a type of white blood cells are produced in large numbers and accumulate in the peripheral bloodstream. The various symptoms that are seen in this symptom are a result of the excess of neutrophils in the bloodstream. There is no definite treatment for CNL. Most cases are treated with the help of various chemotherapy drugs.
What Is Chronic Neutrophilic Leukemia?
According to WHOs (World Health Organization), CNL belongs to a class of myeloproliferative disorders. In this type of blood cancer, there is an overproduction of neutrophils (a type of white blood cells). Neutrophils are responsible for the primary defense of the body against various harmful organisms and foreign bodies. In CNL, a large number of neutrophils are seen in the peripheral bloodstream. It is known as a clonal disorder in which there is an excessive multiplication of identical cells. In clonal disorders, the cells produced are immature and have a varied morphology (shape and size that is not normal). Overproduction and varied morphology are the characteristic features of any cancer.
Neutrophils make up about 40 to 80 percent of the normal bloodstream. Neutrophils always travel to the site of the infection and fight the infection by ingesting the microorganism. They kill the microorganism by secreting an enzyme. The progression of this condition varies from person to person. The treatment and prognosis of the condition depend on the stage of the disease.
What Is the Incidence of Chronic Neutrophilic Leukemia?
CNL is a very rare disease. It was in 2001 that CNL obtained recognition from the WHO. Since then, only 200 cases of CNL have been reported. This condition is mostly seen in older adults, with a median age of 66 years. However, cases have been reported from all ages. CNL affects both men and women equally.
What Are the Symptoms of Chronic Neutrophilic Leukemia?
The most common symptoms of chronic neutrophilic leukemia include the following:
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Anemia: When the body cannot produce enough RBCs, anemia occurs. In the case of CNL, the body cannot produce enough blood cells; the bone marrow is crowded with neutrophils. There is insufficient space for the red blood cells (RBCs) to mature. Anemia can be established with the help of a blood test. Fatigue is the most common symptom of anemia.
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Pain in the Joints: The patient may complain of joint pain. The pain experienced is due to a concept called “leukemic blasts”. The leukemic blast is the condition when the bone marrow is overcrowded with white blood cells. Since the white blood cells crowd the bone marrow spaces, it may cause pain that is similar to bone or joint pain.
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Bleeding and Bruising: There could be more than normal bleeding when the patient is hurt. This happens because there are not enough platelets to arrest the bleeding. This happens as the bone marrow constantly overproduces neutrophils (white blood cells) and other cells are deficient.
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Recurrent Infections: The patient may present with recurrent infections such as fever. The patient may also take longer than usual to heal from common conditions such as the common cold. This happens because the white blood cells that are present are immature to fight any infection. Neutrophils are the first line of defense in our body. When these cells are immature, it is unable to fight any infection.
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Weight Loss: The patient may experience weight loss. This weight loss may be very sudden in nature.
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Night Sweats: Night sweats are the characteristic feature of any cancer. Hence this may be observed in patients with CNL.
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Swelling in the Lymph Nodes: There may be swelling in the neck, armpits, and groin region lymph nodes. This swelling of the lymph nodes occurs when the neutrophils crowd the lymph nodes. The swelling of lymph nodes can occur in the case of any infection also. Hence, it is important to obtain a medical opinion in this case.
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Breathing Difficulties: In leukemia, the white blood cells that are excess in number clump under the lungs (in the thymus gland). This can cause difficulty in breathing. At times, this can also be mistaken for wheezing.
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Pain in the Abdomen: CNL causes pain in the abdomen. This occurs when white blood cells accumulate in the abdominal organs, such as the spleen, liver, and kidneys. This may also be associated with loss and appetite and weight loss. An enlargement of the spleen and liver can also be seen.
How Is Chronic Neutrophilic Leukemia Diagnosed?
The following tests will be performed to determine the occurrence of leukemia. A combination of the following tests will be performed to arrive at the most accurate diagnosis.
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Blood Tests: A complete blood study that includes the complete blood count and the liver function test (LFT) is performed to study the cells, their number, and their morphology (shape and size). The morphology of the cells is studied using a microscope. The blood tests may show an elevated neutrophil count. Blood investigation gives a broad picture of the condition.
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Bone Marrow Aspiration: A sample of the bone marrow tissue is taken to analyze various aspects of the condition. The doctor performs this test with the help of a large hollow needle that is inserted into the bone marrow space. The patient may be sedated with the help of anesthetic agents during this procedure.
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Imaging: Various imaging studies are performed such as X-Ray, CT (computed tomography) scan, and MRI (Magnetic resonance imaging) scan is performed. Such tests help in locating the mass of leukemia and also the cell clusters that may cause abdominal pain and breathing problems.
How Is Chronic Neutrophilic Leukemia Treated?
Chronic neutrophilic leukemia does not have a definite treatment. The treatment of CNL involves the management of the various symptoms rather than bringing a definite cure to the condition. In many cases, the condition may also progress to acute myeloid leukemia (a condition that obstructs the formation of other white and red blood cells). The treatment of leukemia is usually a combination of the below-mentioned methods to obtain the best possible results:
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Drug Therapy: The treatment of CNL involves the usage of various drugs in combinations that are deemed fit by one’s physician. The various drugs that are used in the treatment of CNL include- Chemotherapeutic agents, alpha-interferons, and hydroxyurea.
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Stem Cell Transplant: Stem cell transplantation is a definite cure for CNL. In this treatment, healthy bone marrow is placed in the place of the defective one. Many cases of successful transplantation and remission of the disease have been reported.
Conclusion:
Chronic neutrophilic leukemia is a condition that affects the bone marrow and immature and defective neutrophils are produced. This condition is very rare in nature. It is most commonly observed in older adults over the age of 66 years. Its symptoms may include- joint pain, easy bruising, anemia, fever, weight loss, and night sweats. If diagnosed early in the disease, this condition can have a better prognosis.
