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Everything To Know About Blood Cancer Staging

Blood cancer is a condition that affects the bone marrow and the lymphatic system, leading to the formation of abnormal RBCs, WBCs, and platelets, affecting the normal functioning of other blood cells like nutrition, transportation of oxygen, maintaining normal body temperature and fighting disease-causing organisms. Unlike other types of cancer, blood cancer is not affected by tumour size. It is determined by the number of abnormal cells produced. So it’s staging process is done according to the number of abnormal blood cells released into the bloodstream. Identifying the various stages of blood cancer can aid in the selection of an appropriate treatment method.

Leukaemia  

The stages of Leukemia are determined depending on blood cell counts and the Leukemia cell accumulation of Leukemia cells within organs like the spleen and liver. Each type of Leukemia is staged with a unique system.

1, Acute lymphocytic leukaemia (ALL): It is staged based on the type of lymphocyte, like B and T lymphocytes. The process is as follows; 

B-cell ALL staging-
– Early pre-B ALL: 10% of ALL cases.
– Common ALL: 50% of cases.
– Pre-B ALL: 10% of cases.
– Mature B-cell ALL: 4% of cases.

T-cell ALL staging
– Pre-T ALL: 5 to 10% of cases
– Mature T-cell ALL: 15 to 20% of cases

2, Acute myelogenous leukaemia (AML): It is staged with the help of the French-American-British (FAB) system, which depends on factors like; 
– The number of healthy blood cells
– The size of the leukaemia cells
– The number of the leukaemia cells
– The changes in the leukaemia cell chromosomes.
– Other genetic issues

Important AML stages are as follows;

  1. Undifferentiated AML – M0: Bone marrow cells show no significant signs of differentiation.
  2. Myeloblastic leukaemia – M1: Bone marrow cells show some granulocytic differentiation.
  3. Myeloblastic leukaemia – M2: The bone marrow cells’ maturation is beyond the promyelocyte or early granulocyte stage. 
  4. Promyelocytic leukaemia – M3: The majority of the abnormal cells are early granulocytes, and they contain many small particles and have nuclei of different sizes and shapes
  5. Myelomonocytic leukaemia – M4: Bone marrow and circulating blood have different monocytes and differentiated granulocytes. Monocytes and promonocytes in the bone marrow are greater than 20%. The M4 stage also sees an increase in the number of granular leukocytes called eosinophils.
  6. Monocytic leukaemia – M5: M5 is divided into two categories:
    i, The first subset is characterized by poorly differentiated monoblasts with lacy-like genetic material.
    ii, The second subset sees many monoblasts, promonocytes and monocytes, where the proportion of monocytes is higher in the bloodstream than in the bone marrow.
  1. Erythroleukemia – M6: It is characterized by abnormal red blood cell-forming cells.
  2. Megakaryoblastic leukaemia – M7: Here, the blast cells look like immature megakaryocytes or lymphoblasts. M7 is distinguished by the presence of fibrous tissue deposits or fibrosis in the bone marrow.

3, Chronic lymphocytic leukaemia (CLL): CLL is staged using the Rai system. It considers the following three factors; 
i, Number of lymphocytes in the blood
ii, Degree of lymph node, spleen or liver enlargement 
iii, Presence of anaemia or thrombocytopenia

Rai stage 0 CLL
– High lymphocyte levels are too high
– > 10,000 in one sample
– Blood count normal 

Rai stage 1 CLL
– High lymphocyte level (lymphocytosis)
– Lymph nodes are swollen
– Normal RBC and platelet level

Rai stage 2 CLL
– High lymphocytes number
– Enlarged liver or spleen 

Rai stage 3 CLL
– High lymphocyte number crowds out RBC
– Anaemia 
– Thrombocytopenia
– Swollen lymph nodes
– Enlarged spleen or liver 

Rai stage 4 CLL
– Severe anaemia 
– Severe thrombocytopenia
– Swollen lymph nodes 
– Enlarged spleen or liver

Binet staging system  

Advanced stages of CLL are characterized by the presence of blood disorders. The Binet system evaluates lymphoid tissue instead of relying on blood tests. Binet system staging is as follows;

  • Clinical stage A
    – Swollen lymph nodes
    – Cancer limited to fewer areas

  • Clinical stage B
    – Over three areas of lymphoid tissues are swollen.

  • Clinical stage C
    – Anaemia
    – Thrombocytopenia

  • 4. Chronic myelogenous leukaemia (CML): It is staged based on the total number of diseased cells found in blood and bone marrow cells.

Various stages are as follows; 

Chronic phase CML
 – Early phase of CML
– Common symptoms are fatigue

Accelerated phase CML
– Aggressive phase 
– Result of improper response to treatment 

Blastic phase CML]
– Extremely aggressive phase 
– Blastic refers to over 20% myeloblasts or lymphoblasts.

Lymphoma  

The staging process of lymphoma is the same for both Hodgkin’s and Non-Hodgkin’s lymphoma (NHL). However, there are a few things one should keep in mind; 
– NHL staging is slightly different in adults and children.
– Skin lymphoma is staged differently because they behave differently from other lymphomas. 
– Waldenström’s macroglobulinaemia (WM) is staged differently from the standard staging system, with a score between 0 and 5, based on age and blood test.
– Stages of lymphoma are divided between early (stage 1 or stage 2) or advanced (stage 3 or stage 4). 

Staging of lymphoma in adults

Staging of both Hodgkin’s and Non-Hodgkin’s lymphoma (NHL) is divided into four. 
Stage 1: Lymphoma only in one group of lymph nodes.
Stage 1E: Lymphoma started and is only in one organ situated outside the lymphatic system, called extranodal lymphoma. 
Stage 2: Lymphoma is in two or more lymph nodes and is all on the same side of the diaphragm. 
Stage 2E: It is an extranodal lymphoma. 
Stage 3: It is a condition where both diaphragm sides have lymphoma. 
Stage 4: The most advanced stage is where the lymphoma starts in the lymph nodes and spreads to other organs outside the lymphatic system.

Staging of lymphoma in children 

Stage 1
Lymphoma is only in one group of lymph nodes, but this excludes the chest or abdomen or one organ outside the lymphatic system (extranodal lymphoma), or the spleen.

Stage 2
Lymphoma is found in more than two lymph nodes on the same side as that of the diaphragm. Or it is found in one extranodal body organ and a nearby group of lymph nodes or the gut.

Stage 3
Lymphoma is found in two or more extranodal body organs or lymph nodes above and below the diaphragm or the chest, or the gut. But, this excludes cases where the lymphoma is localised and can be removed by surgery.

Stage 4
Lymphoma is found in the central nervous system or the bone marrow. 

Multiple Myeloma

The staging process of multiple myeloma does not involve the TNM system, but they watch how aggressive the disease is. So, a patient with myeloma and classified under either of the following; 
– Asymptomatic 
– Symptomatic 

Asymptomatic 
It is a condition where the person does not show the signs and symptoms of cancer disease. Such patients are subjected to “active surveillance for smouldering myeloma”, where they are watched closely without specific treatment. But, patients are given the required medication or therapy to stop the spread of the disease. 

Symptomatic 
Patients are showing signs and symptoms of multiple myeloma, and they are classified under the acronym ‘CRAB’. The symptoms under the ‘CRAB’ is as follows;
C – High Calcium levels, leading to a condition called hypercalcemia. 
R – Renal or kidney problems that are diagnosed due to high creatine levels. 
A – Anaemia is the result of low haemoglobin levels in the human body. 
B – Bone damage like lytic lesions (areas of bone damage), osteoporosis (thinning of the bones), and compression fracture of the spine. 
Patients with any of the CRAB symptoms are advised to get treatment for multiple myeloma.  

Other notable signs and symptoms include; 
 – Symptomatic hyperviscosity, which is the abnormal thickening of the blood.
– Amyloidosis.
– Repeated bacterial infections (over two episodes in a year).

Revised International Staging System (R-ISS)

R-ISS is the process used for the staging of multiple myeloma depends on the factors that save a person’s life. The staging process is done based on three factors; 

  1. Measurement of serum albumin
  2. LDH
  3. Serum β2-M
  4. High-risk chromosomes 

Stage I
– β2-M < 3.5 mg/L.
– Serum albumin => 3.5 g/dL.
– Normal LDH levels. 
– No high-risk chromosome changes are found in the myeloma cells.

Stage II
It is neither stage I nor stage III of multiple myeloma.

Stage III
– β2-M > 5.5 mg/L.
– High-risk chromosome changes are found in the myeloma cells. 
– High LDH levels.

In conclusion:

The treatment of blood cancer was initially a challenge because it does not involve isolating the tumour-affected portion from the rest of the body. Cancer cells released into the bloodstream are capable of forming new tumours in other body parts. Today, blood cancer can be treated effectively through methods like chemotherapy and stem cell transplants. The staging process of blood cancer provides insight into the patient’s prognosis as well as which treatment method is the most effective.

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