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Acute Lymphoblastic Leukaemia

Overview

Acute Lymphoblastic Leukaemia (ALL) also known as Acute Lymphocytic Leukaemia is a type of cancer of the blood and bone marrow – the spongy tissue inside bones where blood cells are made. Also referred to as Acute Lymphoid Leukaemia and/or Acute Lymphoblastic Leukaemia, it is characterised by an overproduction of immature white blood cells, called lymphoblast or leukemic blasts. Because the bone marrow is unable to make adequate numbers of red cells, normal white cells and platelets, people with ALL become more susceptible to anaemia, recurrent infections, and to bruising and bleeding easily. The blast cells can then spill out of the bone marrow into the bloodstream and accumulate in various organs including the lymph nodes, spleen, liver and central nervous system (brain and spinal cord). While ALL is the least common kind of cancer affecting adults, among children it is the most prevalent.

Symptoms

The symptoms of Acute Lymphoblastic Leukaemia are caused by a lack of normal circulating blood cells. ALL advances quickly, so people are usually only unwell for only a short period of time (days, or weeks) before they are diagnosed.

Common symptoms of ALL can include:

  • Anaemia due to a lack of red blood cells (RBCs). Anaemia could result in persistent tiredness, dizziness, paleness or shortness of breath when physically active.
  • Frequent and/or recurring infections and slow healing, due to a lack of normal white cells, especially neutrophils
  • Due to a low platelet count, there is increased or unexplained bleeding or bruising
  • Bone and/or joint pain due to the marrow being overcrowded with leukemic cells.
  • Other symptoms could include swollen lymph nodes, chest pain and abdominal discomfort due to a swollen spleen or liver.

Some of these symptoms mentioned above may also be seen in other illnesses, including viral infections, therefore it is important to see the doctor so that proper examination and treatment can happen. In some cases, there may be symptoms and ALL is diagnosed during a routine blood test.

Causes

While the exact causes of ALL still remain largely unknown, it is believed to result from mutations in one or more of genes that are responsible for blood cell development resulting in abnormal growth.

Ongoing research to find possible causes of this damage have identified certain factors that could put some people at an increased risk. These include:

  • Very high doses of radiation either accidentally (nuclear accident) or therapeutically (to treat other cancers)
  • Industrial chemicals such as benzene, pesticides, as well as certain types of chemotherapy used to treat other forms of cancers
  • Certain types of viral infections and the way in which the immune system reacts to the infection could play a role in the development of some types of ALL
  • Certain genetic disorders like Down’s syndrome and Fanconi’s anaemia may have a higher than average risk of developing ALL.

Diagnosis

The World Health Organisation has classified ALL into the following types:

Pre-B-cell ALL:

It accounts for approximately 75-80% of adult cases, ALL arising in the B-lymphocytes which is in the early stages of development in the bone marrow is referred to as precursor B-Cell-ALL or Pre-B-Cell-ALL.

B-cell ALL:

B-cell ALL arises in more mature developing lymphocytes. This form of ALL is rarer and accounts for 3-5% of all adult cases. B-cell ALL is also referred to as Burkitt-like or Burkitt type ALL. Patients diagnosed with B-cell ALL may be treated with similar drugs to those used to treat Burkitt lymphoma.

T-cell ALL:

Approximately 1/4th of ALL cases arise in developing T-cells. This can be further classified as early, mid or late, depending on the maturity of the affected cell. T-cell ALL commonly presents with a high white blood cell count and involvement of the central nervous system at diagnosis.

ALL is diagnosed by examining blood and bone marrow samples in a variety of tests.

Full blood count:

Full Blood Test (FBC) or complete blood count (CBC)is the first step in the diagnosis of ALL. Blood sample will be collected from a vein in the arm and will be investigated in the laboratory. White blood cells may be abnormal leukemic blast cells and the presence of these blast cells in the test suggests ALL. The ALL diagnosis needs to be confirmed by examining the cells in the bone marrow.

Bone marrow examination:

This is performed is the results of the blood tests suggests ALL. A bone marrow biopsy is done to help confirm the diagnosis. A bone marrow biopsy involves taking a sample of bone marrow, usually from the back of the hip bone and examined in the laboratory under the microscope. The sample is examined to determine the number and type of cells present and the amount of haemopoiesis (blood forming) activity taking place there. The diagnosis of ALL is confirmed by the presence of an excessive number of blast cells in the bone marrow.

Further testing:

Once ALL is confirmed, blood and bone marrow cells are examined further using special laboratory tests. These include immunophenotyping, cytogenetic and molecular tests. These tests provide more information about the exact type of disease, the likely course of the disease and the best way to treat it. A small sample of the cerebrospinal fluid (CSF) that surrounds your brain and spinal cord is also collected, during a procedure called a lumbar puncture. This fluid is tested in the laboratory to check for the presence of leukemic cells within the central nervous system.

Chest x-rays and baseline blood tests may also be required, to look at kidney and liver functions.

Treatment

Since ALL progresses quickly, treatment needs to begin soon after ALL is diagnosed. The treatment plan will depend on a number of factors including the sub-type of ALL, the genetic make-up of the leukemic cells, the patient’s age and general health.

Chemotherapy:

Chemotherapy is the primary form of treatment for ALL. A combination of drugs, including steroids, is usually given in several cycles with a rest period of a few weeks in between. The first priority of the treatment is to destroy leukemic cells and induce a remission. This implies that there is no evidence of leukemic cells in the blood and bone marrow and that normal blood cell production and blood counts are restored. In cases, where the risk of relapse is high, patients may be offered even more intensive therapy followed by a stem cell transplant.

Chemotherapy is administered in many different ways to treat ALL and includes intravenously (into a vein), intramuscularly (into a muscle) and in tablet form. To prevent and treat disease in the brain and spinal cord (CNS) chemotherapy is injected intrathecally, directly into the fluid that surrounds these structures. Sometimes, this area is also treated using radiotherapy. In males, radiotherapy may be given to the testes to treat relapsed disease in this area.

Treatment for ALL can be divided into three phases:

  • Induction therapy
  • Post-remission (consolidation) therapy
  • Maintenance therapy

Induction Therapy

Soon after diagnosis an intensive course of treatment begins to bring about, or induce, a remission. If it is resistant or refractory disease, a more intensive form of therapy to treat the disease more effectively may be recommended.

Post-remission (consolidation) therapy

After induction therapy finishes and remission is achieved, more treatment is required to help destroy any leftover disease in your body. This helps prevent a relapse and/or a spread to the central nervous system. consolidation therapy chosen will depend on the estimated risk of relapse.

Maintenance therapy

Maintenance therapy is designed to help keep your disease in remission and prevent it from reappearing (relapsing) in the future. Common maintenance protocols involve chemotherapy tablets — some taken daily and others weekly — and possibly blocks of injections of chemotherapy with courses of corticosteroids. This phase of treatment usually lasts for several months.

Acute myeloid leukaemia

Overview

Leukaemia are cancers that start in cells that would normally develop into different types of blood cells. Most often, leukaemia starts in early forms of white blood cells, but some leukaemia start in other blood cell types. There are several types of leukaemia, which are divided mainly on whether the leukaemia is acute (fast growing) or chronic (slower growing), and whether it starts in myeloid cells or lymphoid cells.

Acute myeloid leukaemia (AML) has many other names, including acute myelocytic leukaemia, acute myelogenous leukaemia, acute granulocytic leukaemia, and acute non-lymphocytic leukaemia. AML starts in the bone marrow (the soft inner part of certain bones, where new blood cells are made), but most often it quickly moves into the blood, as well. It can sometimes spread to other parts of the body including the lymph nodes, liver, spleen, central nervous system (brain and spinal cord), and testicles.

Most often, AML develops from cells that would turn into white blood cells (other than lymphocytes), but sometimes AML develops in other types of blood-forming cells giving rise to different subtypes of AML, as classified by WHO and the FAB systems. The sub-typing depends upon the morphology of cancer cells. Each of these types has a different natural history, presentation and prognosis.

Symptoms

Presently, no screening tests have been shown to be helpful in finding acute myeloid leukaemia (AML) early. AML often develops and causes symptoms to surface fairly quickly. Therefore, the best way to find AML early is to report any possible symptoms of AML right away.

Certain groups of people are known to be at high risk of AML because they have certain blood disorders, such as a myelodysplastic syndrome or inherited disorders such as Down syndrome, or because they were treated with certain chemotherapy drugs or radiation.

AML can show up in the form of one or more of these symptoms:

  • Fever
  • Easy bruising or bleeding
  • Flat pinpoint red spots under the skin caused by bleeding
  • Weakness or feeling tired
  • Shortness of breath
  • Weight loss
  • Loss of appetite
  • Night sweats

Many of these symptoms may occur simply because of a reduction in normal blood cells. So it is important to see a doctor for a conclusive diagnosis.

Causes

Most AML cases can’t be pinned down to exact causes. However, the following risk factors are usually associated with the disease:

  • Smoking, especially after the age of 60
  • Past history of chemotherapy or radiation therapy
  • Having had treatment for childhood Acute Lymphoblastic Leukaemia or ALL
  • Being exposed to radiation
  • Exposure to benzene
  • Having a myelodysplastic syndrome or a bone marrow failure disease
  • Congenital syndromes like Down’s Syndrome
  • Gender (more AML patients are male)

Diagnosis

AML is diagnosed by a simple peripheral smear which requires a drop of blood and examination under microscope. A bone marrow aspiration or trephine biopsy follows. The aspirate helps in identifying blasts and special stains that differentiate other forms of acute leukaemia or from ALL.

A flow cytometry assessment and cytogenetics assessment for karyotype (chromosome arrangement patterns) using peripheral or bone marrow aspirate (preferable) will identify the immunophenotype of the AML and help in ascertaining prognosis.

The patient may need a lumbar puncture to assess spread to brain, if indicated.

Treatment

Chemotherapy is the main treatment for most people with acute myeloid leukaemia (AML). Chemo is often not recommended for patients in poor health, but advanced age by itself is not a barrier to getting chemo. It is usually divided into two phases

  • Induction Soon after diagnosis an intensive course of treatment begins to bring about, or induce, a remission. The goal is to clear the blood of leukaemia cells (blasts) and to reduce the number of blasts in the bone marrow to normal.
  • Consolidation After induction therapy finishes and remission is achieved; more treatment is required to help destroy any leftover disease in your body. This helps prevent a relapse and/or a spread to the central nervous system. consolidation therapy chosen will depend on the estimated risk of relapse.
  • A third, maintenance phase (or post-consolidation), involves giving a low dose of chemo for months or years after consolidation is finished. This is often used to ?treat acute promyelocytic leukemia (APL), but it is rarely used for other types of AML.

Further, in consolidation therapy, once the patient is in complete remission, allogenic transplant may be offered to patients upfront if they have poor cytogenetics, and after first relapse in standard risk patients.

Hematopoietic stem cell transplant is a therapy form, wherein an HLA matched donor’s stem cells are removed by peripheral vein puncture, using an apheresis machine and given to the patient after conditioning therapy. It is superior to autologous transplant, where the patient’s own stem cells are used.

In poor risk patient (those with unfavourable cytogenetics) allogenic transplant confers better survival after inducing first clinical remission than otherwise.

AML is a difficult disease to treat as it is very aggressive, and needs continuous monitoring and follow up. The single most important prognostic factor in AML cytogenetics or the chromosomal structure of the leukaemic cell. Certain cytogenetic abnormalities are associated with very good outcomes (for example, the (15;17) translocation in APML or AML M3). About half of AML patients have ‘normal’ cytogenetics. They fall into an intermediate risk group. A number of other cytogenetics abnormalities are known with a poor prognosis and a high risk of relapse after treatment.

Favourable outcome rates in clinical trials have ranged from 20 to 45%. However, it should be noted that clinical trials often include only younger patients and those able to tolerate aggressive therapies. The overall favourable outcome rate for all patients with AML (including the elderly and those unable to tolerate aggressive therapies) is likely lower. Favourable outcome rates for promyelocytic leukaemia can be as high as 80% – 90%.

Hodgkin’s lymphoma

Overview

Hodgkin’s lymphoma is also known as Hodgkin’s disease. It is a type of lymphoma where cancer originates from the white blood cells called lymphocytes. It was named after Thomas Hodgkin, who first described abnormalities in the lymph system in 1832.

In Hodgkin’s lymphoma, cancer cells spread from one lymph node group to another.

When the cancer cells are observed through a microscope, we get to see multiple RS Cells. The RS Cell, in other words, known as Reed-Sternberg cells which are distinctive, giant cells, found when the patient suffers from Hodgkin’s Lymphoma. The cells usually give the tissues surrounding it, a moth-eaten appearance.

Symptoms

The various symptoms of Hodgkin’s Lymphoma are:

  • Swollen lymph nodes in the neck, armpits or groin
  • Pain in lymph nodes after drinking alcohol
  • Itching throughout the body (Pruritus)
  • Persistent fatigue
  • Coughing, difficulty in breathing, or chest pain arising due to the swollen lymph nodes in the chest pressing the windpipe.
  • Unexplained weight loss
  • Persistent fever
  • Drenching night sweats

The last three symptoms are classified as ‘B symptoms’ which requires aggressive treatment. It is important to note that patients with Hodgkin’s disease may not experience any symptoms or the symptoms may not appear until the advanced stages of cancer.

Causes

Hodgkin lymphoma is caused by a mutation in the DNA of a type of white blood cell called B lymphocytes, although the exact reason why this happens isn’t known. The mutation in the DNA causing them to multiply uncontrollably. The abnormal lymphocytes usually begin to multiply in one or more lymph nodes in a particular area of the body, such as the neck or groin. Over time however, it’s possible for the abnormal lymphocytes to spread into other parts of the body, such as the bone marrow, spleen, skin, lungs and the liver.

Some of the common risk factors identified with Hodgkin’s lymphoma are:

  • Males tend to be at a higher risk than females to suffer the disease.
  • The people in the age group of 15–40 years and over 55 years have a higher chance of the cancer.
  • A family history of Hodgkin’s Lymphoma.
  • Glandular fever, also known as infectious mononucleosis caused by the EBV virus which is directly associated with Hodgkin’s Lymphoma.
  • Weakened immune system due to HIV or AIDS.
  • Prolonged use of human growth hormone.

Diagnosis

Physical Examination:

During diagnosis, the doctor takes into account the medical history of the patient and conducts a physical examination. Here, the doctor checks for swollen lymph nodes in the neck, underarm, groin, spleen and liver. If swelling in the lymph nodes is detected, then some additional tests may be needed to confirm the presence of cancer and determine the extent to which it has spread.

Blood Tests:

Blood tests are performed to check the presence of cancer cells in the blood. Another variant of the standard blood test is conducted to check the erythrocyte sedimentation rate (ESR). In this procedure, blood is collected in a test tube and observations are made to see how quickly red blood cells settle at the bottom of the test tube. Generally, red blood cells settle slowly. A faster rate may indicate the possibility of cancer.

Imaging Techniques:

A chest X-rays shows the lymph nodes in the chest and neck area, which is also known as the starting point for Hodgkin’s Lymphoma. The technique is also used to detect enlarged lymph nodes.

Computer Tomography (CT):

CT scans are generally more accurate than x-rays. They can detect abnormalities in the chest and neck area, as well as reveal the extent of cancer. CT scans are used to evaluate symptoms, understand the stage of cancer and consistently monitor the response to treatment. A CT scan is also often used in detecting lymphomas in the brain, abdominal and pelvic areas.

Positron Emission Tomography (PET):

PET scans combined with CT scans can help doctors clarify the location of cancer. It can also provide information on whether or not an enlarged lymph node is benign or cancerous. PET scans may also help doctors determine how well a patient has responded to treatment, determining if there is any residue of cancer after treatment and if the patient has achieved remission.

Biopsy:

A biopsy of the suspicious lymph node is the most definitive way to diagnose Hodgkin’s disease. A pathologist examines the lymph node sample for the presence of Reed-Sternberg cells or other abnormal features. The type of biopsy performed depends on the location of cancer and how accessible the lymph node is. The doctor may engage in surgery and remove the entire lymph node or a small part of it.

In some cases, the doctor may use fine needle aspiration to withdraw a small amount of tissue from the lymph node to check for cancer cells. Biopsies of bone marrow may also be performed in patients with existing Hodgkin’s disease specifically to determine if cancer has spread to the bone marrow. In such cases, it is termed as Advanced Hodgkin’s Lymphoma.

Stages of Hodgkin’s Lymphoma:

Hodgkin’s Lymphoma can broadly be classified into four stages.

Stage I: In this stage, cancer affects only one lymph node region. There are chances that the cancer has invaded an extra-lymphatic organ but not another lymph node region. Such cases are quite rare in Hodgkin’s Lymphoma.

Stage II: Here, cancer has spread to 2 or more lymph node regions located on the same side of the diaphragm. It is possible that another organ and its regional lymph nodes may also be affected. There are instances when a tumour mass may develop in the chest. In such cases, the size of the tumour can be larger than one-third the diameter of the chest or larger than 10 centimetres.

Stage III: The cancer in the lymph node areas has spread to both sides of the diaphragm.

Stage IV: Here the lymphoma is in its advanced stage where it has spread to 1 or more organs beyond the lymph nodes such as cerebrospinal fluid, liver, bone marrow or lungs.

The treatment depends on the stage of cancer and how far it has spread across the organs. However one of the common symptoms observed is acute pain in the lymph nodes.

Prognostic factors:

In addition to staging, doctors use other prognostic factors to help plan the best treatment and predict how well a treatment will work. For patients with Hodgkin’s lymphoma, several factors can predict the recurrence of cancer and what treatment works best.

Treatment

Treatment options depend on the:

  • Type of Hodgkin’s disease
  • Tumour stage, size, and location
  • Patient’s age and overall health status
  • Presence or absence of “B symptoms” (weight loss, persistent fever and night sweats)

Chemotherapy, Radiation, or a combination of both is the primary treatment options for Hodgkin’s disease. Stem cell transplantation may be recommended for patients who have recurring cancer. During such a stage, high-dose of chemotherapy (along with radiation) is given.

Preparing for Side Effects before Treatment

One of the common side effects is the suppression of the immune system which increases the risk of infections. It is a common practice for patients to be vaccinated against three bacteria namely pneumococci, meningococci and Haemophilus influenza before the actual treatment.

Another side effect is infertility. Patients who may wish to have children in the future should be aware of the risk of infertility after treatment. Men with Hodgkin’s disease may want to consider sperm freezing and assisted reproductive techniques. Women should ask their doctors about the possibility of preserving fertility by taking hormonal drugs called GnRH analogues (responsible for fertility and sex steroids) before undergoing chemotherapy.

Leukemia

Overview

Leukemia (also spelled leukaemia), is a type of cancer of the blood that usually begins in the bone marrow. Leukemia occurs when there is an abnormal increase in under-developed blood cells called ‘blasts’ or ‘leukemia cells’. This abnormality causes the blood cells to grow and divide chaotically. Normal blood cells die after a while and are replaced by new cells which are produced in the bone marrow. The abnormal blood cells do not die so easily. Instead they start accumulating and begin occupying the space intended for normal blood cells, which increases the chances of infection in the body.

The different types of Leukemia are:

CML: A Chronic Myeloid Leukemia (also known as granulocytic leukemia) is a cancer characterized with the abnormal growth of myeloid cells (cells from the bone marrow tissue). In CML, a genetic change in the immature version of the myeloid cells gives rise to the CML cell which can grow uncontrollably from the bone marrow to other parts of the body.

Hairy cell Leukemia: This rare and slow growing cancer occurs when the bone marrow makes excessive number of B-cells (lymphocytes), which are a type of white blood cells responsible for fighting infections. The name ‘hairy cell’ is derived from the hairy appearance of the B-cell observed under a microscope.

Acute Myeloblastic Leukaemia (AML): It is a type of cancer that affects the blood and bone marrow. AML is not a single disease, rather, it is a name given to a group of leukaemias that develop in the myeloid cell line in the bone marrow. Myeloid cells are red blood cells, platelets and all white blood cells excluding lymphocytes.

AML results in an overproduction of immature white blood cells, called myeloblasts or leukaemic blasts. These cells crowd the bone marrow, and prevents it from making normal blood cells. They can also spill out into the bloodstream and circulate around the body. Due to their immaturity they are unable to function properly to prevent or fight infection. Inadequate numbers of red cells and platelets being made by the marrow can cause anaemia, easy bleeding, and/or bruising.

Acute Lymphocytic Leukemia (ALL): It is a type of cancer that affects the blood and bone marrow and is characterised by an overproduction of immature white blood cells, called lymphoblasts or leukaemic blasts. Because the bone marrow is unable to make adequate numbers of red blood cells, normal white blood cells and platelets, people with ALL become susceptible to anaemia and recurrent infections. They also bruise and bleed easily and their healing process is slow. The blast cells can also spill out of the bone marrow into the bloodstream and accumulate in various organs including the lymph nodes (glands), spleen, liver and central nervous system (brain and spinal cord)

T-Cell Acute Lymphoblastic Leukaemia (T-ALL): This specific type of Leukaemia is a variant of ALL (Acute Lymphocytic Leukemia). This cancer affects the white blood cell called T-lymphocytes as opposed to Lymphocytic Leukemia which affects the B lymphocytes. The T-Lymphocytes play an important function of helping the B-lymphocytes make the antibodies to fight infection.

Leukemia is a treatable disease. Most treatments involve chemotherapy, medical radiation therapy, hormone treatments, or bone marrow transplant. The rate of cure depends on the type of Leukemia as well as the age of the patient. It can affect people at any age. About 90% of all Leukemia is diagnosed in adults. It is also the most common cancer type amongst children.

Symptoms

Like all blood cells, Leukemia cells travel through the body. The symptoms of Leukemia depend on the number of Leukemia cells and where these cells collect in the body.

Patients suffering from acute leukemia may encounter symptoms such as headaches, vomiting, sickness, and loss of muscle control or seizure.

Other common symptoms of leukemia include:

  • Poor Blood clotting: As immature white blood cells crowd out blood platelets (crucial for blood clotting), the patient may bruise or bleed easily and heal slowly. This symptom indicates that the patient is in the early stages of leukemia.
  • Affected immune system: The patient’s white blood cells, which are crucial for fighting off infection, may be suppressed or having difficulty in carrying out their function. The patient may experience frequent infections and in some cases his/her immune system may turn against the very healthy cells that they protect.
  • Anaemia: As the shortage of good red blood cells grows, the patient may suffer from Anaemia resulting in difficult or laboured respiration (dyspnea) and pallor (paleness in the skin caused by illness).

Other symptoms:

  • Nausea
  • Fever
  • Night sweats and tiredness
  • Sudden loss of weight
  • Headaches
  • Frequent infections
  • Fatigue
  • Easy bleeding and bruising
  • Swelling or discomfort in the abdomen (arising from a swollen spleen or liver)
  • Pain in the bones or joints

Stages of Leukemia

Stage 0: In the initial stages, there are too many lymphocytes in the body. The symptoms are rarely visible.

Stage I: The lymph nodes start getting swollen due to too many lymphocytes being made.

Stage II: The swelling spreads to the spleen and liver, again due to excessive presence of lymphocytes.

Stage III: In this stage, the patient may experience anaemia since the lymphocytes are crowding out the red cells in the blood.

Stage IV: In the final stage, very few platelets remain in the blood and the lymph nodes, spleen and liver continue to remain swollen.

Causes

There are various causes to the different types of leukemia. Some of the common causes associated with leukemia are:

  • Artificial Ionising Radiation – which can damage living tissue and cause cancer
  • Viruses – HTLV-1 (human T-lymphotropic virus) which are known to cause t-cell leukemia
  • Benzene and Petrochemicals – which contain carcinogenic chemical (cancer causing agents)

Diagnosis

If a patient suffers from symptoms that are associated with Leukemia, one of the following tests will be recommended:

Physical exam: The doctor will check if the skin is pale (due to anemia) and signs of swollen lymph nodes.

Blood tests: The lab does a complete blood count to check the number of white blood cells, red blood cells and platelets. Leukemia causes a very high count of white blood cells. It may also cause low levels of platelets and haemoglobin, which is found inside red blood cells.

Biopsy: The doctor removes tissue to look for cancer cells. A biopsy is the only sure way to know whether Leukemia cells are in the bone marrow. Before the sample is taken, local anaesthesia is used to numb the area. This helps reduce the pain. The doctor removes some bone marrow from the hipbone or another large bone. A pathologist uses a microscope to check the tissue for Leukemia cells. It involves two steps:

  • Bone marrow aspiration: It procedure that involves taking a sample of the liquid part of the soft tissue inside the bones
  • Bone marrow biopsy: The doctor uses a very thick, hollow needle to remove a piece of the bone marrow tissue.

Other tests may include:

  • Cytogenetics: The lab looks at the chromosomes of cells from samples of blood, bone marrow or lymph nodes. If abnormal chromosomes are found, the test can show the type of leukemia the patient is suffering from. For example, people with CML have an abnormal chromosome called the Philadelphia chromosome.
  • Spinal tap:The doctor may remove some of the cerebrospinal fluid (the fluid that fills the spaces in and around the brain and spinal cord). The doctor uses a long, thin needle to remove fluid from the lower spine. The procedure takes about 30 minutes and is performed with local anaesthesia. One must lie flat for several hours afterwards to avoid getting a headache. The lab checks the fluid for Leukemia cells or other signs of problems.
  • Chest x-ray:An X-ray can show swollen lymph nodes or other signs of disease in the chest.

Treatment

Treatments for Leukemia include:

Chemotherapy

Chemotherapy is the treatment of disease with chemo-drugs, designed to kill cancerous cells. This is the main treatment for most types of Leukemia. Chemotherapy is often the primary treatment for children. In case the child suffers from high risk leukemia, then stem cell transplant is prescribed as well. The various side effects of chemotherapy include fatigue, hair loss, infection, nausea & vomiting.

Radiation treatments

Radiation therapy uses high-dose X-rays to destroy cancer cells and shrink swollen lymph nodes or an enlarged spleen. It may also be used before a stem cell transplant.

Stem cell transplant

Stem cells can rebuild the supply of normal blood cells and boost the immune system. Before the transplant, radiation or chemotherapy may be given to destroy cells in the bone marrow and make room for new stem cells. The procedure then involves infusing fresh stem cell back into the blood.

Lymphoma

Overview

Lymphoma is a type of blood cancer that occurs when B or T lymphocytes, the white blood cells, multiply uncontrollably and live longer than they are supposed to. Lymphocytes are white blood cells that move throughout the body in a fluid called lymph. They are a part of the immune system and help protect the body from infection and disease.

Lymphoma may develop in the lymph nodes, spleen, bone marrow, blood or other organs and eventually they form a tumour. Tumours grow and invade the space of surrounding tissues and organs, depriving them of oxygen and nutrients. If abnormal lymphocytes travel from one lymph node to the next or to other organs, the cancer can spread or metastasize. Lymphoma development outside of lymphatic tissue is called extranodal disease.

Symptoms

The primary symptom that manifests when a patient is suffering from lymphoma is the swelling of lymph nodes. This is because the enlarged lymph nodes can encroach on the space of blood vessels, nerves, or the stomach, leading to swollen arms and legs, to tingling and numbness, or to feelings of being full, respectively. The other symptoms that a patient suffers from are; fever, night sweats, weight loss, loss of appetite, fatigue, respiratory distress and itching.

Causes

The following are some of the causes as well as the risk factors of lymphoma cancer:

GENETICS

One of the causes behind lymphoma cancer is the genetic predisposition to the disease that has been inherited from a family member. It is believed that one can be born with certain genetic mutations or a fault in a gene that makes one statistically more likely to develop cancer later in his life.

CARCINOGENS

A carcinogen is a substance or radiation that is an agent directly involved in causing cancer. This could be due to the ability to damage the genome or to the disruption of cellular metabolic processes. They are also responsible for damaging DNA, and aiding in cancer. Exposure to certain pesticides, herbicides and solvents such as benzene has been associated with lymphoma. Similarly, black hair dye has been linked to higher rates of NHL.

Diagnosis

A lymph node biopsy is done in order to diagnose lymphoma cancer. Lymph node biopsy is a partial or total excision of a lymph node which is examined under the microscope. After lymphoma is diagnosed, a variety of tests may be carried out to look for specific features characteristic of different types of lymphoma. These include:

  • Immunophenotyping
  • Flow cytometry
  • FISH testing

Treatment

The classification of lymphoma has a direct effect on the treatment and the prognosis. Classification systems generally classify lymphoma according to:

  • Whether or not it is a Hodgkin’s lymphoma
  • Whether the cell that is replicating is a T cell or B cell
  • The site that the cell arises from

Prognosis and treatment is different for Hodgkin’s lymphoma and between all the different forms of Non Hodgkin’s lymphoma. Another factor that the prognosis depends on is the grade of the tumour, referring to how quickly a cancer multiplies and manifests.

LOW-GRADE LYMPHOMA

Many low-grade lymphomas remain sluggish for many years. In these lymphomas, metastases are very likely. For this reason, treatment of the non-symptomatic patient is often avoided. In these forms of lymphoma, watchful waiting is often the preliminary course of action. The main reason behind delaying the treatment is because the harms and risks outweigh the benefits. If a low-grade lymphoma is becoming symptomatic, radiotherapy or chemotherapy is the treatment option. A person suffering from this can live a near-normal life but the disease remains incurable.

HIGH-GRADE LYMPHOMA

Treatment of some other, more aggressive, forms of lymphoma can result in a cure in the majority of cases. Treatment for these types of lymphoma typically consists of aggressive chemotherapy, including the CHOP or R-CHOP regimen.

Hodgkin lymphoma typically is treated with radiotherapy alone, as long as it is localised. Advanced Hodgkin disease requires systemic chemotherapy, sometimes combined with radiotherapy. Chemotherapy used includes the ABVD regimen.

Multiple myeloma

Overview

Multiple myeloma is a cancer of 1 type of white blood cell. White blood cells fight infections in the body. They are made in the center of your bones, in a part called the bone marrow. When people have multiple myeloma, the bone marrow makes too many of these white blood cells and not enough of the normal blood cells a person’s body needs. This can cause symptoms.

Symptoms

Multiple myeloma can cause many different symptoms. These include:

  • Bone pain or bones that break easily
  • Nausea, vomiting, confusion, or feeling more thirsty than usual
  • Feeling more weak, tired, or short of breath than usual
  • Blurry vision
  • Numbness, tingling, or weakness in the chest, lower back, or legs
  • Getting sick more easily
  • Losing weight without trying to

All of these symptoms can also be caused by conditions that are not multiple myeloma. But if you have these symptoms, let your doctor or nurse know.

Sometimes, symptoms of multiple myeloma can be a medical emergency. For example, it is an emergency if multiple myeloma cells or pieces of broken bone push down on a person’s spinal cord. The spinal cord is the group of nerves that runs down a person’s back. See a doctor immediately if you have:

  • Severe back pain
  • Weakness, numbness, or tingling in the legs
  • No control over your bladder or bowel (that is a new problem)

People with multiple myeloma can get sick from infections more easily than normal. Because of this, it’s important to wash your hands often and stay away from people who are sick. Tell your doctor or nurse right away if you get a fever.

Causes

It’s not clear what causes myeloma.

Doctors know that myeloma begins with one abnormal plasma cell in your bone marrow — the soft, blood-producing tissue that fills in the center of most of your bones. The abnormal cell multiplies rapidly.

Because cancer cells don’t mature and then die as normal cells do, they accumulate, eventually overwhelming the production of healthy cells. In the bone marrow, myeloma cells crowd out healthy white blood cells and red blood cells, leading to fatigue and an inability to fight infections.

The myeloma cells continue trying to produce antibodies, as healthy plasma cells do, but the myeloma cells produce abnormal antibodies that the body can’t use. Instead, the abnormal antibodies (monoclonal proteins, or M proteins) build up in the body and cause problems such as damage to the kidneys. Cancer cells can also cause damage to the bones that increases the risk of broken bones.

A connection with MGUS

Multiple myeloma almost always starts out as a relatively benign condition called monoclonal gammopathy of undetermined significance (MGUS).

In the United States, about 3 percent of people older than age 50 have MGUS. Each year, about 1 percent of people with MGUS develop multiple myeloma or a related cancer.

MGUS, like multiple myeloma, is marked by the presence of M proteins — produced by abnormal plasma cells — in your blood. However, in MGUS, the levels of M proteins are lower and no damage to the body occurs.

Risk factors

Factors that may increase your risk of multiple myeloma include:

  • Increasing age.Your risk of multiple myeloma increases as you age, with most people diagnosed in their mid-60s.
  • Male sex.Men are more likely to develop the disease than are women.
  • Black race.Black people are about twice as likely to develop multiple myeloma as are white people.
  • Family history of multiple myeloma.If a brother, sister or parent has multiple myeloma, you have an increased risk of the disease.
  • Personal history of a monoclonal gammopathy of undetermined significance (MGUS). Every year 1 percent of the people with MGUS in the United States develop multiple myeloma.

Diagnosis

  • Blood or urine tests
  • Bone marrow biopsy – A doctor will take a very small sample of the bone marrow. Another doctor will look at the sample under a microscope to see if cancer cells are present.
  • Imaging tests, such as CT scans, PET scans, MRI scans, or X-rays – Imaging tests create pictures of the inside of the body.

Treatment

People with multiple myeloma often have 1 or more of the following treatments:

  • “Watch and wait”– Some people have a condition called “smoldering myeloma” before they get multiple myeloma. These people do not have any symptoms and might not receive treatment right away. But they do get checked by a doctor regularly. When they start to have symptoms, they will have active treatment.
  • Chemotherapy– Chemotherapy is the term doctors use to describe a group of medicines that kill cancer cells.
  • Steroid medicines– These medicines can kill cancer cells and slow cancer growth. These are  not the same as the steroids some athletes take illegally.
  • Medicines called “immune modulating medicines”– These medicines stop the cancer from growing.
  • Antibodies– Antibodies are proteins in your blood. Your immune system makes them to help your body fight infections. But there are other types of antibodies that are created in a lab and used as medicine. They kill cancer cells by targeting specific parts of the cells.
  • Bone marrow transplant– The bone marrow makes blood cells, including white blood cells. During a bone marrow transplant, a doctor removes some bone marrow from the body. Then, the person gets medicines called “chemotherapy.” These medicines are usually used to kill cancer cells, but they also kill bone marrow cells. After chemotherapy, the doctor puts the bone marrow back into the person’s body.

People with multiple myeloma also get treatment for any symptoms they have. For example, doctors might treat bone symptoms with pain medicines, medicines to stop bone loss, or radiation therapy. Radiation can kill cancer cells.

Non-Hodgkin lymphoma

Overview

Non-Hodgkin lymphoma is a type of blood cancer that includes all types of lymphomas, other than the Hodgkin’s lymphoma. It is a cancer type that starts in the white blood cells, also called lymphocytes, that are part of the body’s immune system. There are two types of lymphomas, which are treated differently, so it’s important to identify the type a patient is suffering from.

Symptoms

Non-Hodgkin lymphomas may present different signs and symptoms, depending on the type of lymphoma and the location of its occurrence in the body. The common signs and symptoms are:

  • Enlarged lymph nodes,
  • Fever,
  • Sweating and chills,
  • Weight loss,
  • Fatigue,
  • Swollen abdomen,
  • Chest pain, and
  • Shortness of breath.

Causes

Researchers have found that non-Hodgkin lymphoma is generally linked to a number of risk factors, but the definite causes of these lymphomas largely remain unknown.

Diagnosis

Doctors and consultants may recommended one of the following diagnostic tests:

  • Imaging test
  • Biopsy
  • Bone marrow examination

Treatment

The main treatments for non-Hodgkin lymphoma are:

  • Chemotherapy,
  • Immunotherapy,
  • Targeted therapy,
  • Radiation therapy, and
  • Stem cell transplant

AL amyloidosis

What is AL amyloidosis?

AL amyloidosis is one type of amyloidosis, which is the buildup of abnormal proteins (called “amyloid”) in the body. These proteins build up in different organs, which can lead to problems.

There are different types of amyloidosis depending on which protein is abnormal. This type involves a type of protein called “light chains.” In AL amyloidosis, the “A” is for amyloidosis and the “L” is for light chains. Doctors also call it “immunoglobulin light chain amyloidosis.” Normal light chains are used to make antibodies, which help the body fight infections. In AL amyloidosis, cells in the bone marrow (the tissue inside your bones that makes blood cells) make abnormal light chains. These abnormal light chains can build up in the kidneys, heart, liver, and other organs.

AL amyloidosis mostly affects older adults, and is more common in men than in women.

Sometimes, AL amyloidosis is related to another problem, such as:

  • Multiple myeloma, which is a cancer of a type of white blood cell called “plasma cells”
  • Waldenström macroglobulinemia, which is a problem with another type of cell made in the bone marrow

Not everyone with AL amyloidosis has one of these other problems. Your doctor will do tests to check for them.

What are the symptoms of AL amyloidosis?

Different people get different symptoms. They might include:

  • Feeling very tired
  • Losing weight without trying
  • Swelling in the feet, ankles, legs, or belly
  • Shortness of breath
  • Numbness or tingling in the hands or feet
  • A large tongue
  • Skin that bruises easily
  • Purple or bruised-looking skin around the eyes
  • Bleeding more than usual, for example, after an injury

AL amyloidosis can affect multiple organs. But in some people, it only affects 1 organ. Commonly affected organs include the:

  • Kidneys – Problems with the kidneys can lead to too much protein in the urine. Kidney problems can also cause fluid to build up in your body, which leads to swelling. Your kidneys might not work as well as they should, which can lead to kidney failure (when they stop working completely).
  • Heart – Your heart might have trouble pumping blood normally. This can lead to symptoms of heart failure, such as swelling or trouble breathing. Some people also have an abnormal heartbeat.
  • Liver – Your liver might be larger than normal.

Is there a test for AL amyloidosis?

Yes. If you have symptoms of AL amyloidosis, your doctor or nurse will do an exam and tests. Some tests look for the abnormal protein people with AL amyloidosis have. Other tests check for problems with your organs.

Tests include:

  • Urine tests
  • Blood tests
  • Biopsy – For this test, a doctor takes a small sample of tissue and then looks at it with a microscope. Your doctor might do a biopsy of your bone marrow plus a biopsy from your belly area. In some cases, they will do a biopsy from a different organ.
  • Imaging tests – Imaging tests create pictures of the inside of the body. Your doctor might suggest imaging tests to look at your internal organs. For example, you might get an echocardiogram (also called an “echo”) to check your heart or an ultrasound to check your liver.

Your doctor might suggest other tests, too, depending on your symptoms. This might seem like a lot of tests. But it’s important for your doctor to know for sure if you have amyloidosis, and which type you have. That’s because AL amyloidosis is treated differently from other types.

How is AL amyloidosis treated?

Treatment for AL amyloidosis includes chemotherapy, bone marrow transplant, or both. There is no cure for AL amyloidosis. But treatment can help reduce symptoms, and help you to live longer.

Before starting treatment, you will need tests. In addition to the tests above, which can tell your doctor that you have AL amyloidosis, you will get other tests. These are so your doctor can see which of your organs are affected, and how much damage there is. They can also check for other problems that might need treatment.

Your treatment might include:

  • Chemotherapy – This is the medical term for medicines that kill cancer cells or stop them from growing. These medicines can also stop growth of the cells that make the abnormal protein in AL amyloidosis.
  • Bone marrow transplant – This procedure replaces the abnormal cells in your bone marrow with healthy cells. It is also called a “stem cell transplant.” When this is done to treat AL amyloidosis, the “donor cells” come from your own body. Stem cells are removed from your blood, then you get chemotherapy to kill the abnormal cells in your bone marrow. After that, your stem cells are returned to your body through a vein. The new stem cells help replace the cells that were destroyed by chemotherapy and grow into healthy bone marrow.

After treatment, your doctor will do regular tests to see if the amount of abnormal protein in your body has gone down. They will also do tests to check if the problems with your organs have gotten better. If not, your doctor might suggest trying a different medicine or combination of medicines.

Waldenström macroglobulinemia

What is Waldenström macroglobulinemia?

Waldenström macroglobulinemia (WM) is a rare type of lymphoma, or cancer of the lymphatic system. The lymphatic system is made up of organs all over the body that make and store cells that fight infection. These infection-fighting cells are also called “white blood cells.” They are made in the bone marrow, which is the tissue in the center of your bones.

In people with WM, the bone marrow makes too many of 1 type of white blood cell. The growth of these white blood cells can cause typical lymphoma symptoms, including swollen lymph nodes, fever, and night sweats. In addition, the white blood cells make a protein called “IgM” or “macroglobulin.” The buildup of this protein in the body can lead to other problems.

WM is also sometimes called “lymphoplasmacytic lymphoma.”

What are the symptoms of Waldenström macroglobulinemia?

Some people with WM have no symptoms for a long time, even years. Doctors call this “smoldering” WM.

When symptoms do happen, they can include:

  • Feeling very tired
  • Weakness or numbness in the hands or feet
  • Losing weight without trying
  • Fever
  • Night sweats
  • Swollen lymph nodes (the lymph nodes are small, bean-shaped organs found throughout the body)

Some people with WM have blood that is thicker than normal. Doctors call this “hyperviscosity syndrome.” This can happen as a result of the extra proteins in the body. Hyperviscosity syndrome is an emergency. It can cause:

  • Bleeding from the nose or gums
  • Changes in vision or hearing
  • Headaches
  • Dizziness
  • Passing out
  • Being unable to move or talk normally

In severe cases, hyperviscosity syndrome can even cause a stroke or coma (a deep state of unconsciousness).

Is there a test for Waldenström macroglobulinemia?

Yes. If your doctor thinks you might have WM, he or she will order tests. They might include:

  • Blood and urine tests
  • Bone marrow biopsy – A doctor will take a very small sample of the bone marrow. Another doctor will look at the sample under a microscope to see if cancer cells are present.
  • Imaging tests – Imaging tests create pictures of the inside of the body.

The results of these tests can tell your doctor if WM or another condition is causing your symptoms.

How is Waldenström macroglobulinemia treated?

It depends. If your WM is not yet causing symptoms, you might not need treatment right away. But you will need to get regular blood tests. It’s also important to tell your doctor right away if you do start having symptoms.

People who have symptoms are treated with medicines. This often includes chemotherapy, which is the medical term for medicines that kill cancer cells or stop them from growing. You might get other medicines, too.

Some people with WM have symptoms due to blood that is thicker than normal. This is treated with something called “plasmapheresis,” or “plasma exchange.” For this treatment, a machine pumps blood from the body and removes the extra IgM protein. Then the machine returns the blood to the body.

Your doctor or nurse might also talk with you about being in a clinical trial. A clinical trial is a research study that uses volunteers to test new treatments.

What happens after treatment?

Treatment does not cure WM, but it can reduce symptoms and help people live longer. Your doctor will do regular exams and tests after you start treatment. This is to see how your body responds to the treatment.

Even if treatment works and your symptoms go away, they will eventually come back. You will continue to see your doctor regularly, probably every 3 or 6 months, for an exam and tests. This way you can start treatment again right away when your symptoms come back, or when tests show that the proteins found in WM are in your body again.

What happens when my WM comes back?

When your WM comes back, you might get the same medicines as you had the first time. Or your doctor might suggest trying different treatments.

What else should I do?

It is important to follow all your doctors’ instructions about visits and tests. It’s also important to talk to your doctor about any side effects or problems you have during treatment.

Getting treated for WM involves making many choices, such as what treatment to have and when. Always let your doctors and nurses know how you feel about a treatment. Any time you are offered a treatment, ask:

  • What are the benefits of this treatment? Is it likely to help me live longer? Will it reduce or prevent symptoms?
  • What are the downsides to this treatment?
  • Are there other options besides this treatment?
  • What happens if I do not have this treatment?