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Thalassemia treatment | BMT

Thalassemia Treatment in India | BMT

Thalassemia treatment in India | BMT

Thalassemia Treatment in India| BMT

Thalassemia is a genetic disorder which affects the hemoglobin in a patient. The conservative treatment consists of recurrent blood transfusions.These recurrent blood transfusion may cause iron overload in the patient and subsequently bone deformity,liver damage and other health complications follow. Bone marrow transplant is considered a viable treatment option for pediatrics thalassemic patients.At CMCS Health, we offer Child Thalassemia treatment In India | BMT in India ,with the best specialist Indian medical doctors at well equipped and well managed treatment centers and hospitals at an affordable and reasonable cost with very good success rates.

What is Thalassemia?

Thalassemia is a medical condition in which the affected person's body produces abnormal hemoglobin. This abnormal hemoglobin gets the excessive and premature destruction of RBCs subsequently causing anemia in the affected person. A person with thalassemia may require frequent blood transfusions depending upon type and severity. We offer comprehensive thalassemia treatment in India | BMT .

What are the causes of Thalassemia?

Thalassemia is a mutation in genes responsible for hemoglobin production. It is a genetic or hereditary disorder, which means the disease is passed on to the patient by the parents who are the carrier of the disorder.

What are the Types of Thalassemia?

Thalassemia can be broadly classified as Alpha and Beta Thalassemia. There are many subtypes of Thalassemia.

Understanding Thalassemia:

Hemoglobin molecules are made up of Alpha and Beta parts. Both Alpha and Beta parts are prone to genetic mutations.

Alpha Thalassemia :

Alpha Thalassemia is more common among the south east Asians , south china,India, Africa and middle eastern countries.

Four Genes are involved in formation of Alpha Hemoglobin chain. A child will acquire two each from both father and mother.

One mutated gene either from father or mother out of the four genes,causes either no symptoms or mild symptoms in child,the child will grow as healthy human being but will be called a silent carrier of Alpha Thalassemia.

Two mutated genes either from father or mother,the symptoms will be mild in child, such a thalassemia is called Alpha thalassemia minor.

Three mutated genes will cause moderate to severe anemia in child. The child will have chronic anemia and will require regular blood transfusions throughout his/her life.This medical condition is also referred as Hemoglobin H disease.

Mutation in all four genes is called Alpha Thalassemia major or Bart hydrops fetalis. Fetuses with mutations in all four genes do not survive or die shortly after birth. Blood transfusion given to fetus with four gene mutations rarely works and have very low success rates.

Beta Thalassemia:

Beta Thalassemia is more common among the people of Mediterranean region. The prevalence is high in North Africa and west asia. Substantial population in Maldives and certain islands in Indian ocean are the careers.

Two genes one each from the father and mother constitutes Beta hemoglobin chain.

One gene mutation causes mild symptoms and are referred as Beta Thalassemia minor.

If both the genes have mutation, the signs and symptoms may vary from moderate to severe.The affected condition is called Cooley's Anemia or Beta Thalassemia Major. Babies born with mutations in both genes are usually born as healthy child but develops complications of thalassemia within two years of birth.Another form of Beta Thalassemia with both genes mutation but with milder symptoms is called Beta Thalassemia Intermedia.

What are signs and symptoms of Thalassemia?

The signs and symptoms of thalassemia depends on it's type.

Signs and Symptoms of Alpha-Thalassemia:

Majority of children with Alpha-Thalassemia or Hemoglobin-H disease are healthy in general.Symptoms may vary from mild to moderate anemia,Fatigue,drowsiness,pale skin or jaundice,abnormally cold hand and feet ,chest pain,frequent headaches,shortness of breath, dizziness and feeling of impending being faint.enlarged spleen and possibly soft to touch and enlarged liver,they may also have deformity of bones,forehead,cheeks ans jaw may overgrow.

Signs and Symptoms of Beta Thalassemia:

Signs and symptoms of Beta Thalassemia ( thalassemia major or Cooley's anemia ) is generally severe and patient may require regular blood transfusions, the symptoms may include jaundice, frequent infections,cold hand and feet,shortness of breath,a general feeling of malice and being tired,poor appetite,skeletal deformity,delayed growth and delayed puberty,iron overload in body that can harm spleen,heart and liver.

How Thalassemia is diagnosed

Different diagnostics tests are recommended by treating doctor,if he/she suspects the patient to have Thalassemia.

  • Complete Blood Count ( CBC) is recommended for hemoglobin levels,as thalassemia patients usually have lower hemoglobin.
  • Microscopic examination: is conducted to ascertain any abnormality in RBCs,Thalassemia patients RBCs are smaller in size as compared to an unaffected person.RBCs with uneven hemoglobin distribution,that gives Bull's eye appearance under the microscope.
  • A reticulocyte Count is done to ascertain how fast RBCs are formed by the bone marrow and released in blood.
  • Hemoglobin electrophoresis separates different molecules in RBCs,allowing identification of abnormal RBCs.
  • DNA Analysis  is done to diagnose thalassemia or to ascertain if the person is carrier of mutated hemoglobin gene cells.
  • Iron tests in blood  are done to ascertain if the patient's anemia is because of thalassemia or iron deficiency,as thalassemic patient's do not have Iron deficiency.
  • Physical examination is conducted to ascertain enlarged spleen.

    Thalassemia Treatment in India|BMT

Treatment for moderate to severe thalassemia may involve frequent blood transfusions.Frequent blood transfusions may cause iron overload in patient's body which in longer run may damage spleen,heart and liver.

Iron Chelation is removal of excess iron from the blood. Patients may be prescribed subcutaneous injections of deferoxamine or oral deferasirox.

Bone Marrow or Stem Cell transplant is a better treatment option for Thalassemia. Chemotherapy cycles given to the patient prior to BMT for destroying abnormal cells and once the patient is in remission phase,bone marrow from a compatible donor preferably a sibling is transplanted in thalassemia patients.

Surgery is also done for some patient with bone deformity and sometimes surgery  removal of spleen and gall bladder is also done.

Dr Vikas Dua is consultant Pediatrics bone marrow transplant specialist at Fortis Memorial Research Institute Gurgaon,India.

Dr Vikas Dua did his advanced Fellowship in Pediatrics Bone Marrow Transplant from National University Hospital Singapore and also did his training in child BMT from world's No.1 institute in pead.BMT ,St. Jude's Children research Hospital Memphis,USA.

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Best Indian bone marrow transplant specialist | CMCS health.

Sickle cell treatment in India | BMT

What is Sickle cell disease?

Sickle cell disease treatment in India | BMT
Sickle cell disease treatment

Sickle cell anemia or sickle cell disease is common in African continent At CMCS Health , we are associated with best Indian Hematologist and BMT specialists for Sickle cell treatment in India | BMT for sickle cell disease patients seeking sickle cell disease treatment in India.

Sickle cell anemia is a severe form of anemia caused by genetic mutation in the Red blood cells. It is a genetic disorder which is inherited by the patient from the two faulty hemoglobin genes from both the parents. Because of the genetic mutation,the shape of RBC ,which is flexible circular disc shaped changes to rigid sickle or crescent shaped rods. Hemoglobin a protein in RBCs,works as a transporter of nutrients and oxygen to cells all across the body.Normal RBCs are disc shaped,with the centers partially scooped out. They are flexible and soft and therefore have the capacity to squeeze through even the tiniest of blood vessels to reach every cell in the body.Due to genetic mutation the RBCs acquire the shape of a crescent moon or Sickle and become rigid and brittle.These Abnormal shaped RBCs which are not flexible get struck in small blood vessels causing slow or blockage of blood flow to other tissues of body and disrupt supply of Oxygen to those parts. Moreover the lifespan of abnormal sickle shaped RBCs is only 10-20 days as compared to 120 days of normal RBCs.Human body keeps on forming new RBCs to replace the old ones,but in a person with sickle cell disease the balance in formation of new RBCs and Destruction of existing RBCs is disturbed. This results in lower RBC count or anemia in a person suffering with Sickle cell disease. Bone marrow transplant in India offers a viable option for Sickle cell treatment in India.

At CMCS Health , we are associated with best Indian bone marrow transplant hospitals for Sickle cell treatment in India.

What are complications of Sickle cell disease?

Sickle cell anemia may have different complications in different people at different stages of disease,but the major and severe complications for a person suffering from sickle cell anemia can be broadly classified as Acute and chronic.

Acute Complication:

Sickle cell crisis:

Sickle cell crisis or VAS ( Vaso occlusive crisis ) manifests as acute severe pain.Sickle cells disrupts blood supply through smaller capillaries resulting in ischemia. Sudden pain across body that may persist from a few hours to a few days.The pain usually occurs in limbs,chest and back.

Fever and infections:

Patients with sickle cell disease are prone to severe bacterial infections because of reduction or absence of splenic function. This may expose the patient to extremely high risk of septicemia and meningitis.Pneumonia is the most common infection ,meningitis,influenza and hepatitis are other infections occurring in sickle cell disease patients.

A Stroke:

Sickle cell disease may cause a a stroke in an affected person because of oxygen deprivation due to blockage in supply of blood to tissue of brain. Some of the sickle cell anemia patients may experience a Transient ischemic attack.

Acute Chest Syndrome:

Pneumonia like complications in a child or adult suffering from sickle cell disease. The reason could be an infection or sickle cells trapped in micro capillaries of lungs.

Hepatobiliary complications:

Hepatobiliary tract abnormalities are common in sickle cell disease patients.They may include cholelithiasis, acute cholecystitis, biliary sludge, and acute choledocholithiasis,acute Hepatic Sequestration and acute intrahepatic cholestasis.

Acute renal failure:

A decrease in kidney functions due to dehydration or obstruction or even because of intrinsic renal disease.

Acute Anemia:

May be because of Splenic sequestration.

Multisystem Organ failure:

A systemic failure of lungs,liver and kidney is a life threatening medical condition for sickle cell anemia patients.

Chronic Complications:

Vision Problems:

A sickle cell disease patient may develop vision problems over a period of time.It may include proliferative sickle retinopathy and vitreous hemorrhage. Complete vision loss is also possible.

Avascular Necrosis:

Avascular or aseptic necrosis can occur when capillaries are occluded by sickled erythrocytes at distal portions of a bone, near a joint, where hypoxia is maximal and collateral circulation is inadequate .The most common site for sickle cell anemia induced avascular necrosis is femoral neck.

Leg Ulcers:

Leg ulcers appear in patients of sickle cell anemia.

Pulmonary Hypertension:

Pulmonary hypertension occur in sickle cell disease patients.

Sickle cell treatment in India | BMT :

Sickle cell anemia patients are usually referred to a Hematologist ( A doctor who has specialized in blood disease management). Treatment of sickle cell anemia may involve determining many factors by the treating hematologist.Treatment of sickle cell anemia includes regular monitoring and follow up with a hematologist,Medications for management of symptoms and complications,pain management,blood transfusions, oxygen supplementation and allogeneic Bone Marrow transplant.

Stem cell or Bone marrow transplant is a viable treatment option for those young sickle cell anemia patients where the diseases condition is severe and can not be managed with conventional methods. A sickle cell anemia child who is having a matching sibling donor is referred for a bone marrow transplant by the hematologist considering all the pros and cons. Risk involved is big but a stem cell or bone marrow transplant is the only cure for sickle cell anemia child or young adult.

Before the transplant, bone marrow cells  are taken from a close matching healthy brother or sister. The patient with sickle cell disease is then treated with chemotherapy and radiation that destroy his or her bone marrow cells. After that, the donated bone marrow stem cells are transplanted in the patient.

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Best Indian BMT doctors - Artemis Hospital - CMCS Health.

Multiple Myeloma treatments in India.

Multiple Myeloma is a diseased medical condition involving plasma cells. Normal plasma cells are found in the bone marrow and form an integral God gifted immune system for a healthy Individual. We offer complete and comprehensive multiple myeloma treatments in India at our state of art and well managed bone marrow transplant unit.

At CMCS Health we are associated with Best Hematologist and BMT specialist from India for cost effective treatment of hematological malignancies in India. We Offer cost effective Multiple Myeloma treatment in India for our Global guests seeking specialized medical treatment in India.

What is Multiple Myeloma:

Multiple Myeloma is a diseased medical condition involving plasma cells. Normal plasma cells are found in the bone marrow and form an integral God gifted immune system for a healthy Individual.

In Multiple Myeloma patients the normal plasma cells are converted into Malignant plasma cells. These Myeloma plasma cells produce large quantities of abnormal immunoglobulins called M Protien.

The large quantities of this abnormal immunoglobulin hampers the production of normal blood cells in Multiple myeloma patients.

Symptoms of Multiple Myeloma:

Although many of the patients suffering from multiple myeloma may not exhibit or feel any symptoms and it is diagnosed because of some lab. tests recommended by a clinician for other health complaints of the patients.

The common symptoms of Multiple myeloma experienced by patients are:

  • Pain in bones ,which could be in all skeleton system but more prominent in skull,back or hips. Patients of multiple myeloma can easily fracture their bones even with a slight stress or injury because of weakening of bone (osteoporosis ) either in the entire skeletal system or at a place of plasmacytoma.
  • A routine CBC recommended by a physician to rule out a health complaint reveals low RBC , WBC and platelets.Low RBCs cause fatigue , a general feeling of malice and not being well, difficulty in breathing and dizziness. Low WBCs will make patient prone to frequent infections and some patient may have pneumonia. Low blood platelet count may culminate  in delayed blood clotting time and excessive bleeding even with a minor cut or bruise .
  • Hypercalcemia or excess calcium in blood may cause extreme thirst and drinking of plenty of fluids and frequent urinating, dehydration,kidney problems,severe constipation, loss of appetite ,a feeling of drowsiness and confusion.
  • Spinal cord compression in multiple myeloma patient because of weakening of bones and collapsing of spinal vertebrae may cause muscle weakness and numbness in legs or sudden severe back pain.
  • Other more severe symptoms may include congestive heart failure, enlarged liver and spleen ,enlarged tongue ,skin changes, persistent diarrhoea and numbness and weakness in hands called carpal tunnel syndrome.

Diagnosis Of Multiple Myeloma:

Once a clinician suspects that a patient is having multiple myeloma ,he recommends certain blood ,urine , a bone marrow biopsy , Xrays ,CT scans and MRIs to confirm the diagnosis.

Multiple Myeloma treatment in India:

The treatment of multiple myeloma depends on staging done by a hematologist. Usually treatment of multiple myeloma consists of chemotherapy and supportive care , radiation therapy ,surgery in certain cases and Bone Marrow or Stem cell Transplant.

At CMCS Health , we offer cost effective multiple myeloma treatments in India with the best of Indian Hematologist and BMT specialists at most well equipped and well managed Indian Blood cancer treatment hospitals.

Dr Pawan Kumar Singh currently heads the Hematology and Bone Marrow Transplantation department at Artemis Hospital in Gurgaon, India.

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Best Indian bone marrow transplant specialist | CMCS health.

Sickle Cell Anemia – complications – treatments.

Sickle Cell Anemia - complications - treatments.

In this blog we highlight Sickle Cell Anemia - complications - treatments.

Sickle cell anemia is a severe form of anemia caused by genetic mutation in the Red blood cells. It is a genetic disorder which is inherited by the patient from the two faulty hemoglobin genes from both the parents. Because of the genetic mutation,the shape of RBC ,which is flexible circular disc shaped changes to rigid sickle or crescent shaped rods. Hemoglobin a protein in RBCs,works as a transporter of nutrients and oxygen to cells all across the body.Normal RBCs are disc shaped,with the centers partially scooped out. They are flexible and soft and therefore have the capacity to squeeze through even the tiniest of blood vessels to reach every cell in the body.Due to genetic mutation the RBCs acquire the shape of a crescent moon or Sickle and become rigid and brittle.These Abnormal shaped RBCs which are not flexible get struck in small blood vessels causing slow or blockage of blood flow to other tissues of body and disrupt supply of Oxygen to those parts.

Moreover the lifespan of abnormal sickle shaped RBCs is only 10-20 days as compared to 120 days of normal RBCs.Human body keeps on forming new RBCs to replace the old ones,but in a person with sickle cell disease the balance in formation of new RBCs and Destruction of existing RBCs is disturbed. This results in lower RBC count or anemia  in a person suffering with Sickle cell disease.

Complications Of Sickle cell anemia:

Sickle cell anemia may have different complications in different people at different stages of disease,but the major and severe complications for a person suffering from sickle cell anemia are:

Acute Complication:

Sickle cell crisis:

Sickle cell crisis or VAS ( Vaso occlusive crisis ) manifests as acute severe pain.Sickle cells disrupts blood supply through smaller capillaries resulting in ischemia. Sudden pain across body that may persist from a few hours to a few days.The pain usually occurs in limbs,chest and back.

Fever and infections:

Patients with sickle cell disease are prone to severe bacterial infections because of reduction or absence of splenic function. This may expose the patient to extremely high risk of septicemia and meningitis.Pneumonia is the most common infection ,meningitis,influenza and hepatitis are other infections occurring in sickle cell disease patients.

A Stroke:

Sickle cell disease may cause a a stroke in an affected person because of oxygen deprivation due to blockage in supply of blood to tissue of brain. Some of the sickle cell anemia patients may experience a Transient ischemic attack.

Acute Chest Syndrome:

Pneumonia like complications in a child or adult suffering from sickle cell disease. The reason could be an infection or sickle cells trapped in micro capillaries of lungs.

Hepatobiliary complications:

Hepatobiliary tract abnormalities are common in sickle cell disease patients.They may include cholelithiasis, acute cholecystitis, biliary sludge, and acute choledocholithiasis,acute Hepatic Sequestration and acute intrahepatic cholestasis.

Acute renal failure:

A decrease in kidney functions due to dehydration or obstruction or even because of intrinsic renal disease.

Acute Anemia:

May be because of Splenic sequestration.

Multisystem Organ failure:

A systemic failure of lungs,liver and kidney is a life threatening medical condition for sickle cell anemia patients.

Chronic Complications:

Vision Problems:

A sickle cell disease patient may develop vision problems over a period of time.It may include proliferative sickle retinopathy and vitreous hemorrhage. Complete vision loss is also possible.

Avascular Necrosis:

Avascular or aseptic necrosis can occur when capillaries are occluded by sickled erythrocytes at distal portions of a bone, near a joint, where hypoxia is maximal and collateral circulation is inadequate .The most common site for sickle cell anemia induced avascular necrosis is femoral neck.

Leg Ulcers:

Leg ulcers appear in patients of sickle cell anemia.

Pulmonary Hypertension:

Pulmonary hypertension occur in sickle cell disease patients.

Sickle cell anemia-Treatments:

Sickle cell anemia patients are  usually referred to a Hematologist ( A doctor who has specialized in blood disease management).

Treatment of sickle cell anemia may involve determining many factors by the treating hematologist.Treatment of sickle cell anemia includes regular monitoring and follow up with a hematologist,Medications for management of symptoms and complications,pain management,blood transfusions, oxygen supplementation and allogeneic Bone Marrow transplant.

Allogeneic Bone marrow Transplant for treating Sickle cell Anemia:

Stem cell or Bone marrow transplant is a viable treatment option for those young sickle cell anemia patients where the diseases condition is severe and can not be managed with conventional methods. A sickle cell anemia child who is having a matching sibling donor is referred for a bone marrow transplant by the hematologist considering all the pros and cons. Risk involved is big but a stem cell or bone marrow transplant is the only cure for sickle cell anemia child or young adult.

Before the transplant, bone marrow cells are taken from a close matching healthy brother or sister. The patient with  sickle cell disease is then treated with chemotherapy and radiation that destroy his or her bone marrow cells. After that, the donated bone marrow stem cells are transplanted in the patient.

Dr Rahul Bhargava is Head of Department-Bone Marrow Transplant and Blood Cancer Treatment at Forts memorial Research Institute,Gurgaon,India.

 

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Thalassemia treatment | BMT

Bone Marrow Transplant for AML in India

Bone Marrow Transplant for AML in India

Bone Marrow Transplant for AML in India-BMT (Bone Marrow Transplant) or Hematopoietic stem cell  transplant is newer medical Intervention for treating hematological malignancies (Blood Cancers) and non malignant medical conditions in children.In this blog,we are discussing the the disease AML,how AML is diagnosed,what are the classific

ation method for AML, treatment choices for AML and Bone Marrow Transplant for AML in India.

What are Indications for BMT in Children?

Bone Marrow or Hematopoietic Stem cell transplant is indicated in certain hematological malignancies as well as non malignant medical conditions in Children.
The major indications for BMT in children because of blood cancers are:
Acute Myeloid Leukemia (AML)
Acute Lymphoblastic Leukemia (ALL)
The non malignant medical conditions requiring BMT are:
Sickle cell disease and Thalassemia.
Aplastic Anemia or other Bone marrow Failure Syndrome.
Immune deficiency diseases,inherited metabolic disorder and others.

Bone Marrow Transplant for AML in India

 

In this Blog we will highlight the need of Bone Marrow Transplant for AML ( Acute Myeloid Leukemias)

Acute Myeloid Leukemia (AML) is a kind of blood cancer.It may consists of multiple disorders involving replacement of healthy bone marrow with abnormal primitive hematopoietic cells.

It may affect both adults and children.AML can affect children of any age and sex.
A subdivision of AML helps the treating doctor chose the best treatment option and ensure better treatment outcome.

AML can be subdivided in a cluster of diseased medical conditions.The most common and widely used classification for sub division of AML is French-American-British system also known as FAB.

Another newer subdivision recommended by World Health Organization known as WHO system is also prevalent.

As per FAB ,AML subdivision is as follows:

M0 - AML with minimal evidence of myeloid differentiation.
M1 - AML without maturation.
M2 - AML with maturation.
M3 - Acute promyelocytic leukaemia (APL)
M4 - Acute myelomonocytic leukaemia
M5 - Acute monocytic/monoblastic leukaemia
M6 - Acute erythroleukemia
M7 - Acute megakaryoblastic leukaemia

Subdivisions of AML as per WHO System:

(Rarely used in pediatrics patients)
AML with characteristic cytogenetic translocations.
AML with multilineage dysplasia.
AML and myelodysplasia syndromes secondary to therapy.
AML not otherwise categorized.

Signs and Symptoms of AML in children: Bone Marrow Transplant for AML in India

As we have discussed in earlier slides that AML is not one
disease but a cluster of associated medical conditions.
The signs and symptoms can also be classified according to diseased medical condition.
Symptoms due to deficiency of normal functioning cells:
Cytopenia,Anemia.Hemorrhage and fever.

Symptoms due to infiltration of abnormal leukemic cell:
Extramedullary infiltration,Mediastinal mass which may cause superior Vena Cava Syndrome or respiratory insufficiency.
Abdominal masses causing pain or obstruction in GI or urogenital tracts.
Gingival hyperplasia.
CNS infiltration.

Causes and factors responsible for pediatric AML:

Causes and factors for AML in children are still being studied.Nothing concrete can be said but the things which
may be linked to AML in children are:
Children with genetic disorders such as downs syndrome or Li Fraumeni Syndrome are considered to be at a
higher risk of developing Pediatric Leukemia.
Although very small,but children with siblings having AML stand a risk of having AML.
Children suffering from Non malignant medical conditions like Aplastic anemia and MDS (myelodysplastic syndrome)
also stand the danger of getting AML.
Link between exposure to radiation,living nearby high voltage power lines and certain chemicals and pesticides are being studied.

Treatments for AML| Bone Marrow Transplant for AML in India

The treatment for AML is guided with intention of destructio  of the leukemic cells and enabling the bone marrow to produce normal cells and function in a normal way.

Chemotherapy is the back bone of AML treatment. A combination of chemotherapeutic or Anti cancer medicines are used for the optimum results and lower side effects.

The treatment of AML is multiphasic.

The first phase is usually called as induction phase. The aim of induction phase is to destroy abnormal leukemic cells. Induction phase involves chemotherapy with combination chemotherapeutic agents for at least for two chemotherapy cycles.

A bone marrow test is conducted at the end of this phase to ensure that the child is free of leukemia.If there is no evidence of leukemia ,the child's condition is referred as being in remission.

Two more cycles of chemotherapy are given to child post the induction phase,to ensure destruction of any left over leukemic cell in blood or bone marrow and prevent any relapse.

Bone Marrow Transplant For AML in India:

Bone Marrow Transplant or BMT is recommended for treatment of AML when the disease has relapsed or the treating doctor feels that the child is at a higher risk for relapse.

Sometimes AML may develop in CNS ,that is spinal chord and brain and in such cases intrathecal chemotherapy is administered to child directly in spinal fluid.Sometimes radiotherapy to brain is also used in conjunction.

 

Dr Vikas Dua is Senior Consultant at DEPT. of Hematology and BMT at FMRI,Gurgaon,India.Artemis Hospital is well equipped and well manged for a complicated medical procedure like BMT.

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