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Writer's pictureAnup Sisotia

Blood Cancer Types and Stages




Blood cancer is divided into three main groups, each group consisting of multiple subtypes. Your medical notes may therefore be a little difficult to understand. Specific blood cancer types are based upon the location and type of their abnormal cells. 

Leukaemia is so named because the cells involved are white blood cells; the prefix leuk refers to white. Because there are many different forms of white blood cells, there are similarly different varieties of leukaemia. For example, cancer of the plasma cells is called myeloma and cancer of the lymphocytes, lymphoma. Confusingly, most publications list leukaemia, lymphoma and multiple myeloma as three different groups, even though they all concern white blood cells. Because even scientific publications use these groups, this article will do the same.


Leukaemia

Leukaemia originates in the undeveloped white blood cells of the bone marrow. Lymphocytic leukaemia’s are not the same as lymphoma. Leukaemia’s develop in the bone marrow and lymphoma originates in the lymph and lymph nodes. Additionally, myeloid leukaemia’s are not the same as multiple myeloma, due to their different types of affected white cell types, even though both types are located in the bone marrow.

Leukaemia is grouped according to the progression of the disease – acute (rapid) or chronic (slowly). Certain types affect very young to teenage children. Paediatric cancer diagnosis is a psychologically and emotionally challenging time for both patient and family. Remedazo and its team members work according to a concentric, holistic approach, understanding the importance of empathy and correct communication. Our team caters for family groups and all ages; we can help to lighten the load during this difficult time. In combination with experienced, renowned paediatric oncologists, you can be sure your child will receive the best treatment in the right environment.


Acute lymphocytic leukaemia (ALL)

Rapidly progressing acute lymphoblastic leukaemia, acute lymphoid leukaemia or acute lymphocytic leukaemia (ALL) can occur at any age but is most commonly associated with children aged between 2 and 4 years old and adults over 45 years old. Treatment in children is highly successful; in adults, the chance of complete remission is lower but still significant.

There are two types of ALL - B cell and T cell. In acute lymphoblastic leukaemia, abnormal lymphoblasts either produce too many B or too many T cell lymphocytes. This disease must be promptly treated. The specific mutation of the cancerous cell must be tested in order to prescribe the most effective treatment.

Causes are usually exposure to radiation (previous therapy) or toxins (pesticides, car pollution, cigarette smoke, chemotherapy drugs), some genetic disorders and perhaps auto-immune processes. The exact causes of ALL are still unknown. 


Acute myeloid (or myelogenous) leukaemia (AML)

Another aggressive form of leukaemia that affects both children and adults is acute myeloid leukaemia or AML; however, this type most commonly affects people of over 75 years of age. As with other acute forms of blood cancer, treatment must be prompt.

While B and T cells help the immune system, AML increases the levels of immature forms of these cells that do not function. Causes are usually exposure to radiation (previous therapy) or toxins (pesticides, benzene, cigarette smoke, chemotherapy drugs), polycythaemia, myodysplastic syndromes, some genetic disorders (Down’s syndrome, chronic myeloid leukaemia) and perhaps auto-immune processes. Because older populations are more likely to develop AML, intensive, high-dose treatments can create more problems than they cure. This means longer courses of therapy with lower doses are advised.

Aggressive blood cancers require prompt treatment by specialist oncologists and haematologists at well-equipped clinics. Remedazo can arrange a long-term stay at accommodation suited to your personal budget within an extremely short time span. We will also assist you in gathering or applying for necessary documentation and plan every aspect of your stay according to your preferences and requirements. This naturally includes arranging consultations with your chosen, highly-experienced specialists, translation services, catering, and transporting you to and from diagnostic, treatment and follow-up sessions at excellent accredited medical facilities.


Chronic lymphocytic leukaemia (CLL)

Chronic blood cancers develop over many years so children are rarely affected. As its full name suggests, CLL is the slow increase of immature (non-functioning) lymphocytes in the blood. Unlike acute forms, we do not yet know why CLL develops.

As this is a chronic cancer, symptoms are few until the cancer is advanced. This also means that when detected at an earlier stage, you may not need immediate treatment. 

H5 Hairy cell leukaemia (HCL)

Hairy cell leukaemia is a subcategory of chronic lymphocytic leukaemia and this blood cancer specifically affects immature B lymphocytes. Under a microscope, these abnormal B cells look as if they have hairs growing out of them.

This form is slow-growing and more likely to affect men over 40 years of age. Like other forms of chronic lymphocytic leukaemia, treatment can wait until symptoms occur


Chronic myeloid (or myelogenous) leukaemia (CML)

While chronic myeloid leukaemia begins as a slowly growing cancer, it can develop into its acute form (AML). CML is most common in populations over the age of 60 and is more likely to affect men, but it is known to occur in both sexes as early as 35 to 40 years of age.

We know exactly what causes CML. It is a genetic disorder caused by a part of a chromosome (Philadelphia chromosome) in blood cell DNA. It is possible to test for this gene by way of a simple blood test or a bone marrow aspiration with biopsy (BCR-ABL test). The results of this test give the necessary information that allows the oncologist to treat you with specific oral drugs. Treatment courses are described later on.

Treatment does not need to be as immediate as treatment for acute forms of blood cancer.


Myelodysplastic syndrome (MDS)

If you are older than 65 years of age you may present with one of various myelodysplastic syndromes. Some are very mild in form, some can produce serious effects. Myelo refers to the bone marrow; in MDS, one or all of the blood cell types cannot develop into mature forms. It is possible for this cancer to progress to AML in a few months or years. This means treatment of those forms that have to potential to cause AML is always necessary, while others are monitored but may not require treatment.


 Lymphoma

There are two types of this rare cancer of the lymphatic system: Hodgkin and non-Hodgkin lymphoma. Non-Hodgkin is the most common. Lymphomas tend to be slow growing but this is not always the case; you may or may not require treatment.

Hodgkin lymphoma affects B-lymphocytes that collect as immature cells in the lymph nodes causing the lymph nodes to grow in size. This enlargement can be visible or palpable in the armpit, neck or groin area. Hodgkin lymphoma is more likely to affect young adults or people over 70 years of age and is linked with the presence of immune disorders, the Epstein-Barr virus, immunosuppressive therapies and family members with Hodgkin. Hodgkin lymphoma is more aggressive than non-Hodgkin lymphoma but can be cured.

Non-Hodgkin lymphoma is linked to chronic infections such as HIV, autoimmune disorders, the Epstein-Barr virus, Helicobacter pylori infections, earlier chemotherapy or radiotherapy and immunosuppressive treatments. Most cases occur in the over-70 age group. Low grade lymphoma is slow-growing; high grade grows rapidly and needs to be immediately treated; however, high grade lymphoma


 Multiple myeloma

The word multiple refers to the many areas of bone tissue that can be affected by this plasma cell cancer. These areas include the spine, the skull, the pelvis and the ribs, any parts of which can become weak and break.

Black men over 60 years of age are more likely to develop multiple myeloma but any gender or race can be affected. If a direct family member has the disease, your risk is higher. It is not always necessary to undergo treatment unless there are associated symptoms. Plasma cells, as previously mentioned, manufacture antibodies that help the body to recognise foreign substances. With multiple myeloma, lots of antibodies are produced but they do not work. These are called monoclonal (M) proteins; they can be detected by way of a simple blood test. Myeloma cells can now be found using a minimal residual disease (MRD) test that are able to detect a single abnormal cell surrounded by of millions of normal ones.


Blood cancer stages

Most cancers are staged based on the size and spread of tumours; however, blood cancer is staged differently because it affects the production of blood cells. This means that staging depends upon the blood cell populations and ratios found in a blood or bone marrow sample.

In addition, oncologists consider a range of broader factors such as your age and medical history when staging blood cancer. 


ALL staging

Staging for acute lymphocytic leukaemia is a complex mix of cell biomarkers, cell counts, age and speed of growth. There are four stages. The first of these is newly diagnosed and untreated which may or may not present with symptoms. The second stage is in remission, or post-treatment ALL. There are no symptoms and blood cell counts have returned to normal. Refractory ALL is acute lymphocytic leukaemia which has not responded to treatment. The final stage is recurrent ALL, referring to the disease’s return after having been in remission. Stages are not listed in order of severity or predicted response to treatment.


AML staging

AML has 8 subtypes – MO to M7 based on the ratio of healthy and unhealthy cells, chromosome changes in mutated cells and other genetic faults. This staging method is called the FAB or French, American and British classification.


MO

AML with minimal differentiation or FAB M0 does not present with large numbers of abnormal cells.


 M1

Acute myeloblastic leukaemia without maturation or FAB M1 on your medical report means that some myeloid cells are not maturing into functional cells but are not completely undeveloped.


 M2

If your report mentions FAB M2 or acute myeloblastic leukaemia with maturation points to a change in the chromosomes of some myeloid cells.


M3

Acute promyelocytic leukaemia or FAB M3 is named after the type of immature white blood cells that have different chromosomes and cannot develop into more mature forms. This type can be treated by a different type of chemotherapy that contains high quantities of vitamin A.


M4

Acute myelomonocytic leukaemia is associated with a very rapid rise in immature myeloblasts to over 20% of the total blood cell volume. This rare type of AML is most often seen in adults over 60 years of age and in children of Asian or Hispanic descent, although anyone can be affected.


M5

Acute monocytic leukaemia refers to the abnormal, immature cell type (monoblast) that is caused by changes in the chromosomes. 


M6

Acute erythroid leukaemia, erthyroleukaemia and mixed forms of these two types increase the numbers of immature red blood cells. This type usually affects elderly males.


M7

Acute megakaryoblastic leukaemia is known to most commonly present in children under 4 years old with Down syndrome and is very rare. This type can significantly lower the levels of mature red blood cells and platelets and lead to easy bruising and bleeding.


CML staging

Because chronic myelogenous leukaemia progresses much more slowly than acute forms, staging tends to look more at the progression first, rather than the cell types; you may therefore see phrases such as chronic, accelerated or blastic on your medical report.

In the chronic phase, less than 10% of your blood cells are abnormal. Your oncologist will probably ask you to come in for blood tests throughout the year but may not start treatment. In the accelerated phase, the percentage of abnormal cells can increase to up to 19%. The blastic phase refers to abnormal cells making up more than 20% of your total blood cells in the presence of symptoms. Treatment for CML is usually started in the accelerated stage. When detected at a later stage, treatment must be started as soon as possible.

A further CML stage is the relapsed stage. This means that, after successful treatment, the numbers of abnormal cells once more begin to increase.


CLL staging

Chronic lymphocytic leukaemia uses yet another staging process – the Binet system. This involves three stages (A, B and C) and refers to the number of affected lymph nodes (adenopathy). All 3 include higher numbers of white blood cells (lymphocytes).

Stage A describes less than 3 groups of enlarged lymph nodes, stage B more than 3 groups of enlarged lymph nodes, and stage C refers to the enlargement of the spleen (or lymph nodes) in combination with low numbers of red blood cells or blood platelets.  

Another method of CLL staging is the Rai system that is categorised into 5 stages:

  • Stage 0: Symptom-free with high lymphocyte levels.

  • Stage I: High lymphocyte levels and swollen lymph nodes.

  • Stage II:  High lymphocyte levels and enlarged liver or spleen.

  • Stage III: High lymphocytes levels that affect the numbers of red blood cells. You blood test results will show that you are anaemic. Possible lymph node swelling or enlarged liver or spleen.

  • Stage IV: Low red blood cell (anaemia) and platelet (bruising) levels due to high lymphocyte levels, possible lymph node swelling and/or large liver and/or spleen


Hodgkin lymphoma staging

Hodgkin lymphoma staging can be confusing as different scales are used for different symptoms and locations. The first method is the Lugano classification with 4 stages. Stage I tells us that only one lymph node or lymph organ is affected. Stage II refers to 2 or more lymph nodes on the same side of the diaphragm. Stage III to lymph nodes or organs on both sides of the diaphragm, and stage IV to wider spread to the liver, lungs or bone marrow.

Additionally, the Lugano classification may end in an A, B or E. A means none of the symptoms listed under B. B includes 3 symptoms of unexplained fever, night sweats and unexplained weight loss over 6 months. The letter E tells us that Hodgkin lymphoma has reached an organ that is close to an affected lymph node.

If the word bulky has been written on your medical report, this means a larger tumour.

The terms resistant and progressive refer to Hodgkin lymphoma continuing to grow even during treatment. Recurrent and relapsed tell us that after responding to previous treatment, the disease has now returned.


Non-Hodgkin lymphoma staging

Non-Hodgkin also uses the Lugano classification described in the previous section. Additionally, the letter S indicates involvement of the spleen. Other stages list low grade (slow growth), intermediate grade (moderate growth) and high grade (rapid growth). All types usually respond to treatment.


 Multiple myeloma staging

Your medical report may include one of 4 multiple myeloma stages: O to IV. Stage 0 or asymptomatic multiple myeloma grows slowly and shows certain proteins called monoclonal proteins and higher levels of plasma cells in blood test or bone marrow results. Treatment is usually not necessary but regular testing is. Stage I means myeloma cells are present as well as a protein called beta-2 microglobulin. Stage II tells us that the multiple myeloma is somewhere between stages I and III. Finally, stage 3 involves high levels of both myeloma cells and beta-2 microglobulin and low levels of another protein called albumin.





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