Blood (Circulatory) System Cancer

Blood Cancer treatment male patient

Blood cancers are systemic, which means they affect the whole blood system.

Leukemia

Facts and figures

Leukemia is cancer that starts in the tissue that forms blood. Most blood cells develop from cells in the bone marrow called stem cells. Stem cells mature into different kinds of blood cells; a myeloid stem cell matures into a myeloid blast which can form a red blood cell, platelets, or one of several types of white blood cells.

A lymphoid stem cell matures into a lymphoid blast; these can form one of several types of white blood cells, such as B cells or T cells. Therefore leukaemias may be lymphoblastic (sometimes referred to lymphocytic) or myeloid. There are four different leukaemias, some of them occur mainly in children and others mainly in adults. 90% of all leukaemias occur in adults.

Childhoood leukaemia

Leukaemia is the most common childhood cancer in the UK and accounts for approximately 33% of all childhood cancers. Higher incidence in boys, male to female ratio is approximately 12:10. The highest incidence occurs between 2 and 5 years of age, particularly acute lymphoblastic leukaemia (ALL) which accounts for 75% of childhood leukaemia.

Most others are acute myeloid leukaemia, with a very small number of chronic myeloid leukaemia but this only about 15 cases a year in the UK. Risk factors include some genetic conditions such as Down’s syndrome, immune system disorders, a sibling having been diagnosed with leukaemia, possibly radiation exposure during early childhood or in utero. There is also some possibility that children whose mothers had a high alcohol intake during the pregnancy may have a slightly increased risk.

Symptoms may include:

  • Increased incidents of fever and infection over short periods of time.
  • Night sweats.
  • Red spots on the skin.
  • Pale and lethargic- may be due to anaemia.
  • Frequent nose bleeds.
  • Poor appetite/ abnormal weight loss.
  • Bone or joint pain.
  • Occasionally shortness of breath.
  • Abdominal pain or discomfort if liver, kidneys or spleen enlarged.
  • Enlarged lymph nodes in the axilla, neck or groin.

Adult leukaemia

Leukaemia is the 11th most common cancer in the UK, accounting for approximately 3% of all new cancer cases. Higher incidence in men, male to female ratio is approximately 14:10. Age related, rises sharply from 45 years of age with an average of 38% being diagnosed in those over 75 years of age and the highest incidence in the 85+ years age group.

Risk factors include exposure to high levels of radiation, previous radiotherapy or chemotherapy, exposure to chemicals such as benzene, smoking, some genetic conditions such as Downs Syndrome and Fanconi anaemia and family history of leukaemia. There are four types of leukaemia in adults:

  • Chronic lymphocytic leukaemia (CLL) – this is the most common adult leukaemia, approximately 3,000 new cases are diagnosed each year in the UK, usually in those over 60 years of age.
  • Acute lymphoblastic leukaemia (ALL) – only about 400 adults are diagnosed each year in the UK, although it is the most common childhood leukaemia. In adults it is most frequently diagnosed in the 15-25 years age group and then in those over 75 years of age.
  • Acute myeloid leukaemia (AML) – this is the 2nd most common adult leukaemia; approximately 2,200 new cases diagnosed each year in the UK, usually in those over 60 years of age.
  • Chronic myeloid leukaemia (CML) is far less common, approximately 680 new cases diagnosed each year in the UK, most commonly occurring in the 40-60 years age group.

Symptoms may include:

  • Increased incidents of fever and infection over short periods of time.
  • Night sweats/unexplained fevers.
  • Pale and lethargic and feeling generally unwell- may be due to anaemia.
  • Abnormal bruising or excessive bleeding.
  • Poor appetite/ abnormal weight loss.
  • Bone or joint pain.
  • Headaches and other neurological complaints.

Diagnosis

Investigations may include:

  • Physical examination.
  • Blood tests.
  • Chest X-ray.
  • Bone marrow aspiration.
  • Bone marrow biopsy.
  • Lumbar puncture.
  • CT scan.
  • MRI scan.
  • Ultrasound scan.

Treatment

Depends on several factors:

  • Type of leukaemia.
  • Stage of disease.
  • Age and general health.

Chronic lymphocytic leukaemia

‘Watchful waiting’ treatment depends on the stage of CLL and in some cases there are no early symptoms so a policy of ‘watchful waiting’ may be recommended.

Chemotherapy

In later stages chemotherapy is usually the main treatment, using fluradarabine, cyclophosphamide and the monoclonal antibody rituximab (FCR), but there are some other options that may be used.

Bone marrow or stem cell transplants

Bone marrow or stem cell transplants are sometimes used, using high dose chemotherapy or radiation to destroy malignant cells in the bone marrow prior to the transplant to put the leukaemia into remission.

Acute lymphoblastic leukaemia (ALL)

Chemotherapy

Chemotherapy will be the main treatment used to treat this form of the disease, along with steroids, the aim being to achieve remission (this is not necessarily a cure). The drugs commonly used are L-asparaginase and vincristine, usually given with the steroid dexamethasone, sometimes methotrexate or daunorubicin may be used. Intrathecal chemotherapy (drugs are administered directly into the cerebrospinal fluid) will also be required to destroy any leukaemic cells that have spread to the brain and spinal cord.

Stem cell transplants

Some will require a stem cell transplant, which involves stem cells being removed from the patient or a donor, then frozen and put into storage. High dose chemotherapy or radiation (total body irradiation TBI) is given to destroy the patient’s bone marrow and then the stored stem cells given to the patient.

Acute myeloid leukaemia (AML)

Chemotherapy

Chemotherapy will be the main treatment used to treat this form of the disease, along with steroids, but it is more intensive than for ALL and is given in 2 phases. The initial phase is remission induction followed by post-remission treatment.

The initial phase usually involves 2 cycles of chemotherapy (often the same drugs as used for ALL) followed by intrathecal chemotherapy to destroy any leukaemic cells that have spread to the brain and spinal cord. A bone marrow biopsy will then be performed and if there are no leukaemic cells found, the child is regarded as in remission and a further course of chemotherapy will be given to destroy any remaining leukaemic cells, aiming to retain remission. Intrathecal chemotherapy will normally be given again after the second course.

Stem cell transplants

Stem cell is used more often in AML than ALL but is still largely limited to children who have experienced relapse.

Adult myeloid leukaemia

Chemotherapy

Chemotherapy will be the main treatment used to treat this form of the disease, and will have two phases, remission induction and post remission treatment. The drugs used may include daunorubicin, doxorubicin and cytarabine. Intrathecal chemotherapy may also be given to treat any leukaemic cells that have, or may have spread to the brain and spinal cord.

Stem cell transplants

Stem cell transplants, using high dose chemotherapy or radiation to destroy malignant cells in the bone marrow prior to the transplant to put the leukaemia into remission.

Other drug therapy

Arsenic trioxide and all-trans retinoic acid (ATRA) destroy leakaemic cells, stop the leukaemic cells from dividing or help leukaemia cells develop into white cells, being used in the treatment of a subtype of AML called promyelocytic leukaemia. Monoclonal antibody is one type of targeted therapy being studied in the treatment of adult AML.

Chronic myeloid leukaemia

Targeted therapy

Patients usually treated initially with imatinib or nilotinib, imatinib usually reduces the number of leukaemia cells in the blood and bone marrow to a level that cannot be detected. Some patients respond initially and then become resistant and a second line drug, dasatinib may be beneficial.

Stem cell transplants

Stem cell transplants are only performed if the disease does not respond to drugs.

Lymphomas

Lymphomas are tumours that develop in the lymphatics, which is part of the immune system. They are classified as either Hodgkin’s lymphomas or Non-Hodgkins lymphomas.

Most lymphomas are Non-Hodgkin’s, accounting for approximately 80%. Hodgkin’s lymphomas contain B lymphocytes, also known as Reed Sternberg cells which are a type of white blood cell that has become malignant. 

The B lymphocytes multiply abnormally and collect in areas such as the lymph nodes. (Although lymphomas are rare in adults, they are the 3rd most common childhood cancer, accounting for 11% of all childhood cancers. The male to female ratio in children is 2:1 and the highest incidence is between the ages of 10 and 14 years. The lymphomas are 50% Hodgkin’s and 50% Non-Hodgkins, their treatment will vary from adults but it is still predominately chemotherapy).

Non-hodgkin’s lymphoma (NHL)

Facts and figures

6th most common cancer in adults in the UK, approximately 12,800 new cases diagnosed each year, which accounts for around 4% of all new cancer cases. Slightly higher incidence in men. Age related, can occur at any age but 60% are diagnosed in the over 60 years age group. There are 60 different types of NHL and they can behave differently.

Most NHL cases are B cell lymphomas, but there are a group of T cell lymphomas that more commonly occur in teenagers and young adults. Most common site is the lymph nodes in the neck, but often found in the liver or spleen and can occur in other body organs. Approximately 33% of patients will have lymphoma in organs outside of the lymphatics.

Abnormal lymphocytes collect in lymph nodes and other places such as the bone marrow and spleen. The causes are unknown but risk factors include a weakened immune system, HIV and AIDS, auto immune diseases, previous Hodgkin’s lymphoma or leukaemia and having a first degree relative diagnosed with NHL slightly increases the risk.

Symptoms may include:

  • Swelling/lump in the neck, groin or axilla, usually painless –most common presenting symptom, particularly lymph nodes in the neck.
  • Night sweats/fever.
  • Unexplained weight loss.
  • Persistent tiredness.
  • Increased incidents of infection.
  • Persistent cough/breathlessness.
  • Persistent itching.

Diagnosis

Investigations may include:

  • Physical examination.
  • Biopsy –tissue sample taken from the lymph node and sent for analysis.
  • Blood tests.
  • Bone marrow biopsy.
  • Liver biopsy.
  • Chest X-ray.
  • Lymphangiogram.
  • CT scan.
  • MRI scan.
  • Laparotomy.

Treatment

Depends on several factors:

  • Stage of the disease.
  • Age and general health.

Monitoring

In some cases of slow growing NHL that are not causing any symptoms, the initial treatment is to monitor the patient and treat at some stage if required.

Chemotherapy

Chemotherapy is the main treatment; there are many different regimens and the drugs used will depend on the type of NHL. The most common is CHOP –Cyclophosphamide, Doxorubin (Doxorubicin Hydrochloride), Vincristine and Prednisolone. This has been found to be highly effect for aggressive lymphomas and has been used for a long time. It is sometimes given without the doxorubicin (CVP) in patients that have pre-existing cardiac conditions.

Radiotherapy

Radiotherapy may be recommended if there are lymphomas cells in only one or two areas of lymph nodes in one part of the body. It may be given after chemotherapy and sometimes this is to reduce the risk of recurrence. Radiotherapy is occasionally used to shrink large lymph nodes for symptom control.

Stem cell transplant

Stem cell transplant may be an option if the lymphoma is in remission but likely to recur, if the lymphoma is in second remission or has not responded to other treatments.

Hodgkin’s lymphoma

Facts and figures

Hodgkin’s lymphoma is a rare malignancy in adults; approximately 1,900 new cases are diagnosed each year in the UK, accounting for less than 1% of all new cancer cases. Higher incidence in men, male to female ratio is approximately 13:10. Age related, can occur at any age but high incidence between 20 and 24 years of age and then again in those over 70 years of age.

The causes are unknown but risk factors are a weakened immune system, exposure to the Epstein-Barr virus or having a first degree relative diagnosed with Hodgkin’s lymphoma, Non-Hodgkin’s lymphoma or Chronic Lymphocytic Leukaemia.

Symptoms may include:

  • Swelling/lump in the neck, groin or axilla, usually painless although sometimes described as aching –most common presenting symptom.
  • Night sweats/fever.
  • Unexplained weight loss.
  • Persistent tiredness.
  • Increased incidents of infection.
  • Persistent cough/breathlessness.
  • Persistent itching.

Diagnosis

Investigations may include:

  • Physical examination.
  • Biopsy –tissue sample taken from the lymph node and sent for analysis.
  • Blood tests.
  • Bone marrow biopsy.
  • Chest X-ray.
  • CT scan.
  • PET scan.
  • MRI scan.

Treatment

Depends on several factors:

  • Stage of the disease.
  • Age and general health.

Chemotherapy

Chemotherapy is the main treatment, sometimes given in combinations with steroids. Most early stage patients will have ABVD chemotherapy – adriamycin (doxorubicin), bleomycin, vinblastine and dacarbizine. There are some other combinations which include chemotherapy drugs and the steroid prednisolone.

Bone marrow and stem cell transplants

If patients have high dose chemotherapy that destroys the bone marrow, they may require a bone marrow or stem cell transplant.

Radiotherapy

Radiotherapy may be used if the lymphoma cells are found in only one or 2 areas of the lymph nodes in the body or may be given in advanced cases to shrink large lymph nodes, usually after chemotherapy.

Biological therapy

Rituximab is a monoclonal antibody that stops lymphoma cells from dividing and growing and may be used in a rare lymphocyte predominant type of Hodgkin’s lymphoma.

Myelomas

Facts and figures

17th most common cancer in the UK, approximately 4,700 new cases diagnosed each year, accounting for approximately 1% of all new cancer cases. Slightly higher incidence in men. The incidence rises sharply form 55 years of age with the highest incidence being in the 85+ years age group, an average of 43% occur in those over 75 years of age.

Risk factors include exposure to high levels of radiation (nuclear power industry), family history of malignant myeloma and occupational exposure to chemicals in rubber production, petrochemicals, farming and painting.

Malignant myelomas are cancers that arise in the plasma cells developing in the bone marrow. In myeloma, the plasma cells become abnormal and excessively multiply and cause problems in multiple places due to the build-up of abnormal plasma cells in the bone marrow.

Symptoms may include

  • In early stage, there are likely to be no symptoms.
  • Abnormal bruising and bleeding.
  • Persistent infections.
  • One pain: 70% of cases present with bone pain, most often lower back and ribs, which is caused by the large number of plasma cells collecting in the bones and damaging them.
  • Hypercalcaemia: symptoms may be nausea, thirst and tiredness.

Diagnosis

Investigations may include:

  • Physical examination.
  • Blood tests.
  • Urine tests.
  • X-rays (particularly if areas of bone pain).
  • CT scan.
  • MRI scan.
  • Bone marrow biopsy.

Treatment

Depends on several factors:

  • The stage of the disease.
  • Age and general health.
  • Treatment will either be intensive (usually younger and fitter) or non-intensive (older or less fit).

Chemotherapy

Chemotherapy is the main treatment, usually melphalan or cyclosphophamide, dexamethasone or prednisolone and either thalidomide or vercade. Patients who receive high dose chemotherapy may have a stem cell transplant.

Biological therapy

Bortezomib is a proteasome inhibitor which blocks the myeloma cells ability to recycle and grow and therefore causes destruction of the cells.

Radiotherapy

Radiotherapy is sometimes used palliatively to treat bone pain.

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