How serious is hairy cell leukemia?

            The human body is made up of many organs and systems that work in a way that enables humans to live and strive. Any changes to this may actually lead to health problems that range from mild to serious. Cancer of the blood is often seen as a serious disease and when a person is diagnosed with one, it can be quite heartbreaking. In this DoctorOnCall’s article, we will be learning about one of the many cancers of blood known as hairy cell leukaemia.

            What exactly is hairy cell leukaemia (HLC)? HLC is a rare type of leukaemia. The name itself derived from the shape of the leukaemia cells that look “hairy” when viewed under a microscope. HLC is known as cancer of the blood and bone marrow. Unique fact about this disease is that it either gets worse slowly or does not get worse at all. HLC often affects adults and approximately 2% of adult patients with leukaemia have this form of disease.

            As the name implies, HLC is a form of leukaemia, it originates from the white blood cells. Specifically, the HLC, the malignant cells are developed from the memory B cell compartment. The B cell compartment is known to make antibodies for the body to help fight against infection. The malignant cells then infiltrate the bone marrow, the spleen and liver. It is not exactly known what is the exact cause that is responsible for such changes but theory of previous exposure to various chemicals may have played a role in development of the malignant cells. Most cases are considered to be derived from gene mutation of the late activated memory B cell.

            People with HLC often have non-specific complaints such as fatigue and weakness. Symptoms such as enlargement of the spleen or lymph nodes may be seen during examination of the abdomen. Other symptoms include easy bruising or bleeding, shortness of breath, pain or feeling fullness below the ribs and painless lumps in the neck, underarm, stomach or groin. Patients might also have a history of frequent infections due to the immune system that is not competitive enough to fight off infections.

            Due to the fact that symptoms are not specific, doctors usually run further tests to narrow down the long list of disease or health problems that share almost the same symptoms with HLC. Blood test such as complete blood count (CBC) can give information on the status of all blood cells in the body. For HLC, 80% of patients have cytopenia. Cytopenia means low level of red blood cells, white blood cells or platelets. Such changes in the blood is caused by the infiltration of the malignant cells affecting the bone marrow function. Less than 10% of patients do have severe pancytopenia (low level of red and white blood cells with platelets). Doctors will also run additional blood tests such as blood smear in which a sample of blood is checked for the “hairy” cells. Bone marrow aspiration and biopsy may be performed in certain cases. This procedure is done by inserting a small hollow needle into the hipbone or breastbone to attain small samples of bones, bone marrow or blood. Immunophenotyping is a test that helps to identify the hairy cells with the use of markers that are expressed on all malignant cells. BRAF gene testing may also be done in which BRAF gene mutation is often found in patients with HCL.

            Once diagnosis is established, treatments given will depend on several factors such as the number of the malignant cells, spleen condition and if there was recurrent leukaemia after previous treatment. Although current treatment is not considered to cure the disease, therapies available do help control the disease. Common medications are cladribine and pentostatin, which is a 2-purine analog, and are part of anticancer drugs. This type of medication is typically given to those with symptoms and significant cytopenia. Those without any symptoms are monitored closely for progression of the cancer every 3 to 6 months. Immunotherapy such as interferon may be given as treatment to boost or restore the natural body immune’s system. Splenectomy may be considered in massive spleen or severe pancytopenia. Prescription of antimicrobials as means of prevention against infection may be considered.

            Now you have known a lot more about HLC, you might wonder how serious this disease can be. Due to the slow growing nature of the disease, the disease can be kept under control for many years with treatment. This is known as remission where the disease is not active. Remission can be evaluated through symptoms and blood samples. Relapse can be expected to last for 11 years after first treatment. Doctors also expect most people will have a normal lifespan such as other healthy people. In general, 95 out of 100 people with HLC survive for 5 years or more after they are diagnosed. Thus, it is safe to say while HLC is a serious disease due to the fact it is leukaemia, patients are at high chance to live at least for the first 5 years after diagnosis.

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