Dr. Santanu Sen

AREA(S) OF INTEREST:

Bone Marrow Transplantation, Thalassemia & Sickle Cell Disease , Leukaemia, Lymphoma & Blood cancers, Brain tumours, Neuroblastoma and other Paediatric Tumours

PROCEDURES PERFORMED:

Paediatric Bone Marrow Transplantation – Allogeneic, Autulogous and Haploidentical Transplants done for Thalassemia, Sickle Cell Disease, Aplastic Anaemia, Relapsed Leukemias and Lymphomas, Resistant cancers, Relapsed/resistant Ewings Tumour and Neuroblastoma; Consultation services for various benign haematological conditions such as Thallassemia, Sickle Cell Disease, Haemophilia, Idiopathic Thrombocytopenic Purpura, Anaemia and other blood disorders; Consultation services for Malignant conditions such as Leukemia, Lymphoma, Brain tumours, Bone tumours such as Ewings Tumour and Osteosarcoma, Wilms tumour and other abdominal tumours, Neuroblastoma, Rhabdomyosarcoma and other soft tissue sarcomas; Expertise in Haemophagocytic Lympho Histiocytosis (HLH) and Langerhans Cell Histiocytosis (LCH)

FELLOWSHIPS:

• Paediatric Oncology, Royal Victoria Infirmary, Newcastle, UK
• Stem Cell Transplantation, Newcastle General Hospital, UK
• Paediatric Oncology, Royal Marsden Hospital, London, UK

• Examiner: Royal College of Paediatrics & Child Health, UK
• International FACT/JACIE accredited BMT Unit Inspector
• Top Doc Mumbai 2019
• Top Doc Mumbai 2020
• Indian Icon Award Winner 2015

• Dr. Sen has 20+ years of experience in the field of Paediatric Oncology and has been doing Bone Marrow Transplants for the last 18 years
• Dr. Sen has worked in Royal Marsden Hospital in London, which is one of the world's best cancer hospitals, treating patients with leukemia, lymphoma, brain tumours and other paediatric cancers and all pediatric bone marrow transplant patients there.
• He has the most experience in undertaking half Matched (Haploidentical) transplants in the region and extensive experience in transplants for blood disorders.

KDAH Experience:

At Kokilaben Dhirubhai Ambani Hospital he has treated cases of paediatric cancers including leukemia, lymphoma, brain and other tumours. He is the In Charge of the Paediatric Stem Cell Transplantation program which has to its credit transplanting the smallest baby in the world (transplanted for Neuroblastoma at the age of 4 months) and the youngest thalassemia transplant in India at 9 months. With tie ups with world renowned institutions such as Royal Marsden Hospital in London, Royal Victoria Infirmary in Newcastle and Karolinksa Institute in Sweden he ensures that the latest technology and therapy is available for all his patients.

• Low-cost matched sibling bone marrow transplant for standard-risk thalassemia in a limited-resource setting; Ramprakash S, Agarwal R, Dhanya R, Marwah P, Soni R, Yaqub N, Fatima Itrat, Zhara T, Gooneratne L, Williams S, Khalid S, Sen S, Kanwar V, Faulkner L, Pediatric Hematology Oncology Journal (2018), doi: 10.1016/j.phoj.2017.12.002
• Impact of Splenomegaly on Outcomes of Full Matched Sibling Bone Marrow Transplant Among Children with low Risk Thalassemia; Stalin Ramprakash, Rajat Agarwal, Rakesh Dhanya, Priya Marwah, Rajpreet Soni, Naila Yaqub, Fatima Itrat, Lallindra Gooneratne, Senani Williams, Sadaf Khalid, Santanu Sen and Lawrence Faulkner Blood 2017 130 :1944
• Role of G-CSF priming of donor marrow prior to transplant in low risk HLA matched sibling transplant in children with severe thalassemia; Stalin Ramprakash, Rajat Agarwal, Rakesh Dhanya, Priya Marwah, Rajpreet Soni, Naila Yaqub, Fatima Itrat, Lallindra Gooneratne, Senani Williams, Sadaf Khalid, Santanu Sen and Lawrence Faulkner Pediatric Hematology Oncology Journal; Volume 2, Issue 2, Supplement, 2017, Page S22
• Voriconazole induced periostitis: A rare entity Santanu Sen, Shruti Sidavasan, Sameer Tulpule, Tanu Singhal Pediatric Hematology Oncology Journal; Volume 2, Issue 2, Supplement, 2017, Page S30-S31
• Haploidentical bone marrow transplants in a pediatric and young adult population: A tertiary centre experienceSantanu Sen, Sameer Tulpule, Nisha Iyer, Tanu Singhal, Rajesh Sawant, Pankhi Konger Pediatric Hematology Oncology Journal; Volume 2, Issue 2, Supplement, 2017, Page S24-S25
• Rejection of paternal vs maternal fully matched bone marrow grafts in children with thalassemia.; Ramprakash S, Agarwal RK, Dhanya R, Sedai A, Kumari A, Parmar L, Srinivas R, Kakulamari VR, Marwah P, Soni R, Williams S, Rathnayake W, Sen S, Tulpule S, Faulkner L. Bone Marrow Transplant. 2017 Nov;52(11):1585-1586.
• Panayiotopoulos syndrome presenting with status epilepticus and cardiorespiratory arrest: a case report. Mujawar QM, Sen S, Ali MD, Balakrishnan P, Patil S. Pediatric Emergency Care. 2011 Aug;27(8):754-7
• National outcome of paediatric oncology patients requiring intensive care ; Santanu Sen, Sucheta Vaidya, Jonathan Round, Andrew Pearson; Pediatric Blood & Cancer, October 2007 49(4):(p 490)
• Lineage switch at relapse in a case of stem cell leukaemia Santanu Sen, Mary Taj; Pediatric Blood & Cancer, October 2007 49(4):(p 518)
• Paediatric Index of Mortality (PIM) scores under predict deaths in Paediatric Oncology patients;Santanu Sen, Sucheta Vaidya, Jonathan Round, Andrew Pearson; Pediatric Blood & Cancer, October 2007 49(4):(p 584)
• Outcome of paediatric oncology patients requiring intensive care S. Sen, T. Morris, S. Vaidya, J. Round. Archives of Disease in Childhood 2007;92(Suppl I):A94
• Hybrid chemotherapy in two children with acute leukemia of ambiguous lineage. Velangi MR, Reid MM, Sen S, Skinner R, Taj MM. Pediatric Blood & Cancer, June 2006, 46(7): (p 833)
• Propofol anesthesia is an effective and safe strategy for pediatric endoscopy. Perera C, Strandvik GF, Malik M, Sen S, Paediatric Anaesthesia. February 2006;16(2):220-1.

Professional Memberships:

• Examiner – Royal College of Paediatrics & Child Health, UK
• Examiner – College of Physicians & Surgeons, Mumbai
• West Zonal Executive Council Representative, Pediatric Hematology Oncology Chapter, Indian Academy of Paediatrics
• Executive Council Representative, Indian Academy of Pediatrics, Mumbai Branch
• Member, International Society of Paediatric Oncology
• Member, Children Cancer & Leukemia Group, UK
• Member, American Academy of Paediatrics
• Member, American Society of Clinical Oncology
• Member, European Society of Blood & Marrow Transplantation
• Member, Indian Society of Hematology & Transfusion Medicine

Dr. Santanu Sen: Tumour in 1 week old baby

Naveen(name changed for privacy of patient) was only 1 week old when his doctors told his devastated parents that he has a large tumour in his tummy.

A lot of detailed tests including ultrasonography and CT scan of his abdomen confirmed the suspicion that he has a neuroblastoma, a tumour that originates from the supra-renal gland on top of the kidneys.

Further tests and a biopsy of the tumour showed that though the tumour was limited only to the original site with no distal spread (metastasis), it was positive for ‘Myc-N’, a genetic marker which signifies that it was a very aggressive tumour that needs intensive treatment.

Chemotherapy was started from 1 month of age and he underwent 8 cycles of intensive chemotherapy over the next 3 months and at the age of 5 months had surgery to remove the tumour.

But even this was not enough as this aggressive tumour needs further high dose therapy followed by stem cell transplantation for its treatment.

The problem was that at this young age of 6 months and weighing only 6 kg, a transplant was a difficult proposition and very few institutions has the expertise for such a procedure.

The BMT team at KDAH, which has the significant experience of doing paediatric, transplant was uniquely suited for this difficult task. Dr Santanu Sen, who had extensive experience of paediatric transplants in UK said that the fact that they have a complete multidisciplinary team with full time consultants makes it a safe place for such difficult procedures.

The first major challenge was collecting the stem cells from the patient for the transplant. A special machine called a cell separator is used to centrifuge and filter out the stem cells from the blood but using it on such a small baby was challenging. This was because almost the entire child blood volume had to be cycled very rapidly and constantly through the machine. In fact the machine had to be primed (filled) with a separate blood unit from a donor prior to use as otherwise the childs own blood volume would have been inadequate to even start the machine. In fact the child blood was cycled through the machine once every 10-15 minutes for 6 hours to get enough stem cells for the transplant.

The stem cell collection was done in the Paediatric Intensive Care Unit with both the Paediatric and Neonatal Intensive Care Consultants in constant attendance to monitor the baby. In fact, the Paediatric Cardiac Intensivist was at the bedside constantly monitoring the babys heart throughout the procedure to ensure the child safety.

Successful collection of stem was done with no untoward events and a second collection was again done the next day.

This was the first time that such a procedure has been done on such a small child in India and internationally, less than five such cases has been reported in the literature.

The stem cell transplantation was successfully carried out and the child showed signs of recovery within 10 days which also is very early (usual is 14-21 days). In fact the child was discharged in a stable condition on the 18^th day after the transplant which also is a record as usually it takes upto 4 weeks before a child is discharged.

Dr. Santanu Sen: First every bone marrow transplant for sickle cell disease successfully performed at KDAH

What is Sickle Cell Disease?

It is a genetic disease that causes severe disability and even death in many patients. The disease causing geneis passed from generation to generation in a pattern of inheritance called autosomal recessive inheritance. This means that both the mother and the father must pass on the defective form of the gene for a child to be affected.

Affected children suffer from anaemia, severe episodes of pain in chest, abdomen or joints, painful swelling of hands and feet, frequent infections, shortening of height, blindness, stroke or even death if not treated properly.

It happened because in sickle cell disease, the red blood cells become rigid and shaped like sickles. These irregularly shaped cells can get stuck in small blood vessels, which slows and blocks blood flow and oxygen to parts of the body.

If only one parent passes defective gene to the child, that child will have the sickle cell trait. Their blood might contain some sickle cells, but they generally don't have symptoms. But they are carriers of the disease, which means they can pass the gene to their children.

Is it dangerous?

It is a disease that causes severe debility and misery. Children suffer severe infections with frequent episodes of severe incapacitating joint pains and even paralysis due to strokes. In fact, in India, one of the ICMR surveys found that 20% of children with sickle cell disease die by the age of 2 years! In some affected areas, 30% of all affected patients die before reaching adulthood!

What is the extent of problem in India?

In India, there is a huge sickle population in parts of Maharashtra, Gujrat, Madhya Pradesh, Chattisgarh, Bihar and some parts of West Bengal, Kerala and Tamil Nadu. Valsad and Nagpur have large population of patients suffering from the disease. It is estimated that upto 40% of the population in the affected regions carry the defective gene. Exact numbers for the disease in India is not know, but is is estimated that worldwide 300,000 children are born every year with sickle cell disease.

What is the treatment?

Treatment includes medicines to reduce pain, blood transfusions to treat the anaemia and antibiotics to treat infections. A Bone Marrow Transplantation offers the only known cure for the disease.

What are the results?

A BMT done early from an unaffected brother or sister who doesn’t have the genetic defect offers an excellent outcome with long term cure. Finding a suitable donor can be a problem as siblings may also be affected and in India finding an unrelated matched donor is very difficult.

Any recent advances?

Recent advances in BMT has made it possible for patients to have a transplant from brothers and sisters who are not fully matched with the patient. This new type of BMT called Haploidentical BMT offers the chance of a cure for a vast majority of patients.

What about our patient?

Manish Bhoi, a 16-year-old patient, suffered from repeated painful swelling of hands and feel and had multiple admissions for infections, joint swellings and severe headaches. He was diagnosed to have Sickle Cell Disease and even with optimum medical therapy continued to suffer with recurrent painful episodes and repeated hospital admissions. He was also admitted with suspected stroke! In fact, he became so distressed at the prospect of a lifelong battle with the disease that he got severe depression and needed extensive psychiatric therapy.

Understanding that a BMT offered him the only possibility of a cure, we started the process of identifying a suitable donor. HLA typing to identify a suitable match was done for the family, and though his sister was disease free, she unfortunately was only a half-match, as were his parents. A search was initiated to identify any possible fully matched unrelated donors, but unfortunately none were found.

As any further delay would only increase disease related problems and would make a transplant even more complicated, we decided to proceed with a half-matched (Haploidentical) transplant with his sister.

Unfortunately, we found that his sister had very high levels of antibodies directed against her brother’s cells (Donor Specific Antibody – DSA). This makes BMT extremely difficult as there is a high possibility of graft failure. Hence we initiated a novel innovative protocol to reduce the antibody levels before initiating the transplant protocol.

The BMT was done using his sister stem cells and he engrafted early by Day +11. He was kept under close supervision and discharged after a successful transplant.

What is unique about this case?

First successful transplant for Sickle Cell Disease in Western India and that too using a Haploidentical donor.

Previous cases were mostly from Chennai, Delhi and Bangalore and were almost all using a fully matched sibling.

Our BMT program can bring about a paradigm change in the lives of all patients with Sickle Cell Disease.