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Leukaemia - Learning About Blood Cancer

Leukaemia is a type of cancer that effects blood and its components. Once the marrow cell undergoes a leukaemia change, the leukaemia cells may grow and survive better than normal cells. Over time, the leukaemia cells crowd out or suppress the development of normal cells.The rate at which leukaemia progresses is different with each type of leukaemia. Leukaemia can broadly be divided into rapidly growing or acute leukaemia and more indolent, chronic leukaemia. A proper knowledge about this disease is essential as it helps patients and his care givers to cope better with this disease. After diagnosis and treatment, a large number of people with leukaemia live many good, quality years.

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Acute Lymphoblastic Leukaemia (ALL)

It is a rapidly growing blood cancer which requires urgent management. Treatment of ALL has undergone a sea change in last 30 years, from a certain death in few days in 1940s to about 80-90% chance of cure now, treatment of ALL has improved remarkably over the years.There are few important things to know about this disease

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Symptoms

Symptoms are usually due to replacement of normal blood cells by abnormal ' blast ' cells

  • Anaemia - It is a condition where there is low number of red cells. As a result, patient has fatigue and shortness of breath.
  • Neutropenia - It is a condition where number of white cells, especially type of cells called neutrophils, are reduced, so that body is unable to fight against infections.
  • Thrombocytopenia - It is a condition, where there is low number of platelets, causing easy bruising or bleeding, which can be severe at times.
  • Pancytopenia - All blood cells are reduced.
  • Other signs and symptoms include pain in arm, legs or hips, enlarged lymph nodes, liver, spleen, pale skin, prolonged bleeding from cuts, tiredness, vomiting, unexplained weight loss.

Treatment

Patients with ALL require treatment as soon as possible after diagnosis because of fast disease progression. Treatment approach depends upon individual ' s subtype, risk factors, any infection at the time of diagnosis. Generally treatment can last between one and a half to three years. Treatment plan involves either of following options :

  • Chemotherapy is the treatment of choice for most of childhood and adult ALL. It involves using multiple anti cancer drugs in various combinations. Treatment generally lasts for 3 years and cure rates of 80-90% can be achieved. It involves replacing diseased blood of patient from a healthy donor, who can be patient's sibling or from worldwide registries (Matched unrelated donor transplant).
  • Patient undergoing BMT are at very high risk of infections, therefore special 'hepafiltered' rooms are required. It is a very specialized treatment, available at only select centres of India.

Acute Myeloid leukemia (AML)

ALL is most aggressive of all blood cancers and can be a difficult disease to treat. Active research is going on in clinical trials to study new approaches to treat this devastating disease. For most people who have AML, as with other cancers, there are no obvious risk factors as to why they developed this disease. Patient should start treatment as soon as possible in a centre experienced in treating acute leukaemia.

Symptoms

The signs and symptoms of AML are common to other, less serious illness. There is usually a short history of 5-7 days before symptoms become more severe. Symptoms resemble those mentioned above for ALL except that lymph node, spleen, liver enlargement is less common and bleeding , fever is more prominent. In addition AML can present as mass of tumour cell in any part of body (myeloid sarcoma).

Treatment

Most AML patients, particularly patients with high WBC counts, need treatment soon after diagnosis because of rapid disease progression. After diagnosis, initial aim is to get disease under control or in remission. Long term goal is to cure the disease.

  • Chemotherapy
  • Chemotherapy followed by BMT
  • Exact choice depends upon many factors like age, type of disease etc.
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Chronic Myeloid Leukaemia (CML)

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In contrast to acute leukaemia mentioned above, chronic leukaemia are more indolent and slow growing. CML is the most common type of leukaemia in India. CML when it was discovered, was untreatable and resulted in death in few months to years. However with a new drug called as imatinib these patients have almost normal life span .

Symptoms

Symptoms can be follows :

  • Big spleen -fullness in abdomen
  • Hepatomegaly - enlarged liver
  • Weakness, fever, fatiguability, bleeding can be seen in advanced stages

Treatment

Treatment is with tablet imatinib, which has to be taken life long.

Chronic Lymphocytic Leukaemia (CLL)

CLL is the most common leukemia of the west and second most common in India. It is usually diagnosed on routine blood test (CBC) in otherwise healthy patient, where there is increase in a specific type of cell(Lymphocyte). Most of the CLL patients do not require treatment, which is initiated only when CLL becomes symptomatic.

Most common indications to start treatment are :

  • Weakness, Fever
  • Large Lymph Nodes
  • Large Spleen
  • Rapidly Increasing Lymphocyte Count
  • Anaemia or Low Platelet Count
  • Even if treatment is required, it is effective and long term disease control can be achieved.
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Advances in Hematology

Numerous advances have been made in the field of hematology that have significantly improved the lives of patients with hematologic disorders. In the last 50 years alone, substantial strides have been made in the research, treatment, and prevention of blood diseases. Listed here are just a few, but those not mentioned are no less important. As past experience has shown us, even some of the most basic research taking place today has the potential to lead to promising life-saving treatments in the future.

Major Advances in Leukemia

  • Cure of Childhood Acute Lymphoblastic Leukemia (ALL)
    ALL was fatal for every child who was diagnosed in the 1960s, but today, after new combinations of drugs were developed and aggressive treatment of the brain and spinal fluid were incorporated, approximately 80 percent of children with the disease are cured. Even in relapsed ALL CAR T cell therapy and Antibody therapy (Blinatumomab) has revolutionized treatment.
  • Targeted Therapy ("smart" drugs) for Chronic Myelogenous Leukemia (CML)
    In the 1950s the only treatment for CML was radiation of the spleen, granting patients about 30 months of survival. The identification of the mutation in the BCR-ABL caused by a specific chromosomal translocation (when a piece of one chromosome breaks off and attaches to another) led to the development of imatinib, a gene-targeting drug that is the paradigm of a new generation of "smart" drugs. Imatinib is the first drug that has been designed to be so specific that it targets and corrects only the molecular defect that leads to CML (unlike chemotherapy, which targets all rapidly dividing cells, both diseased and normal), sparing patients most of the toxic side-effects that usually come with chemotherapy. Treated with this non-toxic oral drug, more than 75 percent of patients diagnosed with CML now achieve a durable complete cytogenetic remission. This development introduced a new way to think about treating cancer patients. With the disease in long-term remission (inactive, but not necessarily cured) many CML patients can now expect a normal life span.  Small number of patients who relapse on this therapy can be offered second line treatment like Nilotinib , Desatinib.
  • Targeted Therapy for Acute Promyelocytic Leukemia (APL) APL was once described as the most malignant (rapidly worsening) form of acute leukemia. Leukemia cells contain molecules that disrupt normal function of the hemostatic system (which controls the flow of blood) and, as a result of initial treatments that triggered the release of these molecules, many APL patients bled to death before the chemotherapy they were given had a chance to work. A simple and effective therapy for this process was established in the 1980s that allowed chemotherapy drugs to kill the leukemia cells without initiating the deadly reaction. In combination with chemotherapy, targeted treatment has significantly improved survival in patients with APL and raised remission rates to about 85 percent. Today, the treatment of APL has become a model for treating cancer with targeted therapy
  • Improved Survival in Acute Myeloid Leukemia (AML)
    More than 13,000 new cases of AML will be diagnosed in this year. Without treatment, AML can progress quickly and become fatal in just a few months. However, over the past 30 years, new targeted therapies and aggressive chemotherapy have dramatically increased the rate of patients who stay in remission for years or are cured. Today, the initial remission rate is approximately 80 percent.

Major Advances in Lymphoma and Lymphoid Malignancies

  • Cure of Hodgkin Lymphoma
    Today, many patients survive Hodgkin lymphoma. Advancements in radiation therapy and chemotherapy have helped transform this once fatal disorder into a routinely cured disease. More than 80 percent of patients are cured after primary treatment, and the success of treating Hodgkin lymphoma has created hope for curing other forms of cancer.  Even relapsed Hodgkin Lymphoma have a good chance of cure with newer therapies like stem cell transplant, immunotherapy like Brentuximab, etc.
  • Immunotherapy for Non-Hodgkin Lymphoma
    The incidence of non-Hodgkin lymphoma has been increasing since the 1970s, making it the fifth most common cancer. Recent years have seen the development of promising immunotherapy treatments (which stimulate the body's immune system to fight disease) for patients, increasing survival rates. Studies have shown that five-year survival without recurrence of the lymphoma signals a high likelihood of cure. During the 1960s only 31 percent of patients reached that benchmark.Today, that number has more than doubled to 64 percent.
  • Improved Therapy and Survival in Multiple Myeloma
    There will be more than 19,000 new cases of multiple myeloma this year, and approximately 34 percent of patients survive five years after diagnosis. Unfortunately, there is not yet a cure; however, thanks to advancements in stem cell transplantation and treatment therapies, patients are living longer, healthier lives than they were 30 years ago. Recent Medicines like Daratumumab , Pomalidomide , Carfilzomib have improved prognosis of even relapsed and refractory Myelomas.

Major Advances in Genetic Diseases

  • Effective Prenatal Diagnosis of Abnormal Hemoglobins
    Sickle cell disease and thalassemia are among the most common genetic diseases . Before 1983, there was no national neonatal screening program to detect either disorder. Today, newborns with these diseases greatly benefit from early detection. Those diagnosed with sickle cell disease have a greater rate of survival than children diagnosed later in life.
  • Major Advances in AnemiaDevelopment of Diagnostic Techniques to Prevent Stroke in Sickle Cell Disease
    Despite progress in understanding the causes of sickle cell disease, the health of patients with this disease was largely ignored until the 1970s. During the 1980s and 1990s, several studies worked to improve the quality of life for these patients. In 1998, transcranial screening allowed doctors to identify sickle cell patients at risk for stroke and treat them with blood transfusions to prevent stroke.

Major Advances in Anemia

  • Cloning of Erythropoietin and Development of Recombinant Epo Clinical Use
    More than 50 years ago, patients suffering from anemia due to chemotherapy, chronic kidney disease, AIDS, or other disorders were dependent on frequent blood transfusions in order to replenish their red blood cells. In between transfusions, when their red blood cell counts dropped, they would suffer from weakness, fatigue, and shortness of breath. In the 1950s and 1960s scientists conclusively showed that a hormone in the body, erythropoietin, was responsible for regulating red cell production. Twenty years later, researchers developed a nearly identical, synthetic version of that hormone, known as epoetin alfa (Epo), that could be mass-produced and administered by injection under the skin. Today, millions of patients worldwide have benefited from Epo, one of the most widely used drugs created through recombinant DNA technology. Epo has allowed them to live active lives without the inconvenience and risk of repeated transfusions.
  • Immunotherapy for Aplastic Anemia
    Aplastic anemia is a rare and serious condition that can be traced back to 1888. In the past, severe aplastic anemia was fatal to nearly half of all patients regardless of treatment, but with the development of immunosuppressive therapy combined with other treatments, survival rates have substantially improved, and more than 60 percent of patients experience successful responses to immunotherapy. It is considered as treatment of choice in patients of aplastic anemia who are older than 40 years of age.

Major Advances in Stem Cell Research

  • Development of Successful Hematopoietic Stem Cell Transplantation
    Today, hematopoietic stem cell transplantation (HSCT) is an important approach for treating blood and bone marrow disorders, as well as certain types of cancer. The earliest work with HSCT began in the 1950s. By the 1960s this treatment was successfully used in patients with end-stage leukemia. Subsequent research in this area has led to improved transplantation techniques and improved survival rates for a number of diseases. Today it offers only chance of cure to a large number of non cancerous blood disorders like Aplastic anemia , Thalassemia , storage disorders etc.