Blood cancer, 2 seemingly innocuous syllables of the English vocabulary. Easy to say, simple to understand. And yet, these innocent sounding words are sufficient to make a grown man break out in a sweat, and send shivers down one’s spine.
Why should a diagnosis of Blood Cancer evoke so much of fear and anxiety? When you receive a diagnosis of blood cancer what exactly does it mean? Does it indicate imminent death or is it now a disease that in modern times can be effectively treated?
Blood Cancer Information:
Understanding Blood Cancer Diagnosis:
Blood cancer is a very broad term that encompasses a number of conditions, some of which are aggressive, while the majority are indolent and slow growing. What is important to understand is that irrespective of the diagnosis, all can be treated. A significant proportion can be cured completely if treated appropriately.
Any abnormality arising from the bone marrow comes under the blood cancer heading. In simple terms- this can mean a Leukemia, Lymphoma or a Myeloma. However, to a lay person, it is usually the aggressive acute leukemia that is thought of, when given a blood cancer diagnosis.
How to diagnose blood cancer? Who can be affected? How can we treat it? Will you die if you have blood cancer? These are the questions I will answer in this blog. We will also discuss blood cancer diagnosis and treatment.
Types and Stages of Blood Cancer:
The principal types are the Acute Leukemias, the Chronic Leukemias, B and T cell Lymphomas and Multiple Myeloma.
The staging is determined based on the molecular profiling of each blood cancer.
Blood cancers, especially the acute leukemias- acute myeloid leukemia and acute lymphoblastic leukemia can affect both men and women of all age groups. Children and the elderly are considered to be especially prone to this disease, being at the extremes of age.
What you must remember is that if you are found to have blood cancer, it does not mean that you are going to die soon. All blood cancers can be treated and the majority can be cured. A fraction of the cancers, if not cured, can be controlled, allowing you to lead a normal life.
Cause: the cause remains unknown in most patients. Prior radiation received as treatment for another cancer, exposure to ionizing radiation-as from a nuclear fall- out, certain chemotherapy drugs, smoking, alcohol, tobacco consumption, a positive family history in relation to some of the blood cancers, certain DNA mutations which may be inherited or develop spontaneously, are some of the causative factors known.
Symptoms and Signs Blood Cancer :
A blood cancer, may remain quiescent for an extended period of time, without any symptoms. The affected individual may continue to lead a normal life without realizing that he is harbouring a malignancy. Blood cancer diagnosis may occur on a routine blood test (commonly occurs in CML- Chronic Myeloid Leukemia and Chronic Lymphocytic Leukemia), or on a routine sonography or body scan (seen in Lymphomas).
An elevated creatinine or a deranged calcium level- indicating an altered renal function could point towards a diagnosis of a myeloma.
Acute Myeloid and Acute Lymphoblastic Leukemias can also remain asymptomatic in the initial stages.
A high or low WBC count, a low or an excessively high hemoglobin, a high or low platelet count, changes in the differential WBC count, abnormal cells seen on a peripheral smear examination, are all indicators of a possible leukemia and must be investigated further.
In the advanced stages, a complete bone marrow suppression, resulting in pancytopenia can occur. This is possible in all blood cancers, but classically seen in Leukemia, especially the acute leukemias.
A routine CBC can pick up all the above abnormalities.
Symptoms that must arouse suspicion are– a low or a high- grade fever that does not respond to antibiotics, it may come intermittently or remain continuously. Fever that does not settle within a few days must be investigated further with a CBC.
An evening rise of temperature, night sweats, weakness, fatigue, bony pains, feeling breathless on mild exertion, repeated infections, a loss of appetite, weight loss, a feeling of fullness are other symptoms that need evaluation.
A drop in platelet count can cause gum bleeding, bleeding in urine or stools or a bleeding beneath the skin resulting in small purple spots called petechiae. Minor trauma can cause bruising. Cough with blood- tinged sputum can occur if there is a concurrent chest infection.
A drop in hemoglobin can cause anaemia leading to weakness, fatigue and breathlessness.
Lymph node swellings can develop, especially in Lymphomas. These would be seen as a sudden onset swelling in the neck, arm-pit or the groin. A single or multiple swellings may develop depending on the lymph nodes involved.
Liver and spleen enlargement occur in both leukemias and lymphomas. An abdominal discomfort followed by abdominal heaviness and pain occur as their size increases.
Both lymph node swellings and liver/spleen enlargement can be seen on a sonography, CT scan or PET-CT scan.
A multiple myeloma must be suspected when there is persistent bony pain, for example- back pain, that is continuous, and does not subside with routine treatment. Minor trauma leading to a fracture, or a spontaneous fracture occurring without any fall or trauma must arouse suspicion.
A sudden onset renal failure, an unexplained increase in creatinine indicating renal dysfunction, a sudden inability to pass urine are all potential symptoms of a myeloma.
A reduction in immunity with an increase in vulnerability to infections is classical of all blood cancers, especially in leukemias and multiple myeloma.
Serum immunoglobulin levels are found to be deranged in Myeloma.
Blood Cancer Diagnosis and Treatment:
Blood Cancer Diagnosis:
When a blood cancer is suspected based on symptoms or blood tests, the most important thing is not to panic. Instead of surfing the internet and trying to figure out what to do next, the best thing to do is to consult an expert, in this case- an experienced Haematologist or Medical Oncologist.
They are qualified to treat blood cancers and are the appropriate people to provide further guidance. The suspicious blood report or scan must be given to them for their opinion. The patient must meet the Haematologist/ Medical Oncologist as well. A detailed physical examination and an assessment of the patient’s overall condition is necessary before planning further tests.
False Impression:
There are situations like a viral infection which can also cause a bone marrow suppression, resulting in a mistaken diagnosis of a leukemia. Atypical cells and abnormal lymphocytes are also seen in the CBC and peripheral smear examination of such patients. A detailed history is therefore necessary before making a diagnosis. Such patients will present with a high- grade fever which will persist for an extended time.
However, the majority will settle with supportive care. Bone marrow suppression eventually resolves over the next few weeks, once the fever subsides. Closely monitoring these patients with regular CBCs will bring out the correct picture.
Tuberculosis is another disease that can often be misdiagnosed as a Lymphoma. The reverse is also true. A lymph node swelling which is a Lymphoma, may be mistaken for Tuberculosis and treated as such. An excision biopsy of the concerned lymph node and a detailed histopathology with immunohistochemistry (IHC) studies on the biopsy is necessary for a correct diagnosis.
Further Tests For Blood Cancer Diagnosis:
Once the consultant confirms the suspicion of a blood cancer, further evaluation is required depending on the type of blood cancer suspected.
If a leukemia is suspected, a bone marrow aspiration and biopsy are required. An extended panel of tests are then performed on the bone marrow sample. These are Immunophenotyping or Flow Cytometry, FISH studies, Cytogenetics/Karyotyping and Molecular evaluation. These are necessary to classify the leukemia into acute or chronic types.
These are further subdivided into acute myeloid, acute lymphoblastic, chronic myeloid and chronic lymphoblastic leukemias. The treatment of each is separate, and depends on the results of the above tests.
A routine work up like liver function and renal function tests, a chest Xray, sonography of the abdomen and pelvis, a cardiac evaluation with an ECG and a 2D Echo are required in all blood cancers before treatment initiation.
Any underlying infections must be aggressively treated with antibiotics before beginning treatment.
If a Lymphoma is suspected an excision biopsy of the concerned lymph node is necessary. A Tru-cut biopsy is insufficient and can result in a mis-diagnosis. A PET-CT Scan must be performed to assess the extent of the lymphoma. A bone marrow aspiration & biopsy are also required to check for bone marrow involvement by the lymphoma.
Lymphomas can be either T or B cell in origin. These are further subdivided into multiple categories, with the treatment for each being different. Follicular, Diffuse Large B cell, Mantle cell, Anaplastic and Burkitt’s Lymphomas are some of the more frequently occurring ones.
Histopathology and IHC studies will determine the type of Lymphoma. The molecular studies will then define the precise line of treatment required.
Treatment for every blood cancer is now customized for every patient, based on its unique molecular profile. Every patient will receive a treatment suitable for his cancer, age and physical characteristics. The days of ‘one size fits all’, is now history.
Multiple Myeloma also requires a bone marrow aspiration and biopsy for diagnosis. FISH studies, molecular tests and Cytogenetics/Karyotyping tests are performed on the bone marrow samples.
Blood and urine tests consisting of protein electrophoresis, immunofixation and free light chain assays must be carried out. Serum immunoglobulins and beta 2 microglobulin tests must be performed on a blood sample. The results of the molecular studies determine the treatment required.
A full body PET-CT Scan is helpful in assessing the extent of bony involvement by the Myeloma, and the presence of any soft tissue or organ involvement.
Emotional Impact Of Diagnosis:
Getting diagnosed with Blood Cancer is a scary and emotional situation for any patient. It is natural to have thoughts of death come up in one’s mind. No matter how strong the person, a blood cancer diagnosis is sufficient to shake up an individual.
Feeling anxious and scared regarding the treatment is also expected.
During this period, the support from family and friends, and especially from the treating doctor, is very important, in reality, this forms the backbone of the treatment.
Understanding the blood cancer diagnosis, the treatment, its duration, all help in coping with the diagnosis.
Staying positive mentally is essential. Keeping the mind diverted by engaging in other activities during treatment is also important.
Discussing and talking to family members while blood cancer treatment is ongoing goes a long way in maintaining one’s resolve. This ensures that negative thoughts do not come up and hinder the ongoing therapy.
Surfing the internet and trying to self-treat the blood cancer is a strict no. All this does is it increases the fear psychosis and results in undesired confusion.
Above all, maintaining faith in the treating doctor is essential. The doctor will eventually be the source of strength for the patient.
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Treatment Options for Blood Cancer
In the 21st century, the treatment of blood cancer has undergone a sea change. No longer is it a universally lethal disease that cannot be treated.
Today, with the medical advances we possess, blood cancers can be treated and most can be cured, while the slow growing, indolent cancers can be controlled.
Modern treatment allows us to customize treatment for each patient, ensuring they receive the most effective therapy, with minimal side-effects, that allows them to continue their daily activities, while receiving cancer care.
The treatment for each blood cancer differs, depending on whether it is a Leukemia, a Lymphoma or Multiple Myeloma. These are further divided into subtypes with each having a different treatment protocol based on its molecular profile and patient characteristics.
Leukemias:
Acute Myeloid and Acute Lymphoblastic Leukemias require aggressive, intensive therapy to effect a cure. High dose chemotherapy in combination with oral targeted therapy is the backbone of the treatment. Molecular characteristics and specific DNA mutations detected in the concerned patient determine which drugs would form a part of the treatment.
A number of patients will attain a cure with chemotherapy and targeted therapy alone. Patients who carry high risk mutations, those who relapse post treatment and those who are refractory to first line treatment- will require a matched Allogenic Stem Cell Transplant for a cure.
Chronic Myeloid Leukemia:
These patients carry a good prognosis. Current treatment regimens cannot provide a cure. However, the disease can be controlled, allowing patients to lead a normal life. Treatment involves an oral tablet to be taken daily. Imatinib is the drug commonly used in the first line. Relapsed and refractory patients experience an aggressive form of the disease, and require an Allogenic Stem Cell Transplant for a cure.
Chronic Lymphocytic Leukemia/Lymphoma:
These patients generally remain under close observation during the initial years of the disease. Since a cure is not possible, and the leukemia is slow growing, treatment is only initiated in case of symptoms like fever, loss of appetite, weight loss, a drop in hemoglobin or platelets or in case of organ compromise. Targeted therapy in the form of tablets is the initial treatment used, with molecular profiling guiding the treatment decisions. Chemotherapy upfront is not required.
Multiple Myeloma:
Initial treatment involves a combination of oral and injectable targeted therapy. Molecular profiling and presence of high/low risk DNA mutations determines the treatment line. Bortezomib, Lenalidomide, Dexamethasone and Denosumab are the drugs used initially. Following remission, younger patients are offered an autologous transplant while the older patients are continued on oral Lenalidomide as a maintenance. Myeloma cannot be cured, only controlled. Daratumumab is a new drug, used in the first line in patients who carry high risk mutations.
B and T Cell Non-Hodgkins Lymphoma and Hodgkins Disease:
These are classified into numerous subtypes on the basis of molecular profiling. The treatment of each subtype is different. Treatment usually involves a combination of chemotherapy and targeted therapy. Relapsed and refractory patients require a Stem Cell Transplant. Rituximab is the targeted therapy commonly used. Some of the lymphomas, like Diffuse large B cell can be cured. While slow growing lymphomas like Follicular lymphoma can only be controlled.
Living with Blood Cancer
Adjusting to blood cancer treatment and learning to live with it can be difficult. In many patients, life long treatment is needed. The acute leukemias require aggressive, intensive therapy which is stressful and physically draining.
Support from family and close friends is essential during these times. Understanding the illness and the treatment, its duration, the side-effects and how to tackle them, all help in getting through this difficult period smoothly. Sharing a good rapport with the treating doctor is important during this entire period.
Avoiding smoking, tobacco, alcohol, exercising regularly if possible, consuming a diet rich in vegetables and fruits is essential. Crowded places must be avoided to reduce the risk of infection. A mask must be worn when out of the house as protection against air-borne pathogens.
Support & Resources:
The treating physician would be the go-to person to obtain reading material with reference to the cancer. The physician would also be able to connect the patient with other blood cancer patients. There are multiple online support groups available, depending on the specific type of blood cancer diagnosed.
Conclusion:
Blood cancer in the 21st century is a disease that can be treated. Patients must not be afraid. Staying positive and trusting the treating physician is the key. Do not be afraid to ask questions, staying informed makes the treatment easier and allows it to progress smoothly.
Being diagnosed with a blood cancer is not a death sentence any longer.
