L J Ravi 13-year-old boy with a rare genetic blood disorder cured at BGS Gleneagles Global Hospital
The patient was diagnosed with inherited bone marrow failure syndrome along with blood cancer was successfully treated with a novel approach
Bengaluru : A 13-year-old child who had bleeding and an extremely low platelet count of 4000 was brought to BGS Gleneagles Global Hospital around a year ago. Clinical evaluation and a complete assessment of the patient indicated that the patient had Fanconi anemia (inherited form of aplastic anemia)which had transformed to a devastating blood malignancy known as Acute Myeloid Leukemia (AML).
Fanconi anemia (FA) is a rare genetic disorder that affects the bone marrow as well as many other organs.The bone marrow fails to produce healthy blood cells and platelets in people with Fanconi anemia.Blood problems and several types of cancer are more likely to occur in FA patients than compared to general population. FA patients usually also have certain structural abnormalities, most commonly short stature.
“Following the child’s diagnosis of acute myeloid leukemia (AML), a cancer of the blood and bone marrow, the family was counseled regarding the diagnosis, potential treatment options for putting AML into remission followed by the necessity of ALLO HSCT, as well as the extreme high risk associated with treatment and the poor prognosis, including mortality,the parents were explained about the novel approach of tackling his AML,as bringing him into remission was very important before proceeding to the curative option of transplant.” Added Dr. Prerana S Nesargi, Associate Consultant, Paediatric Oncology and Haematology, BGS Gleneagles Global Hospital.
Allogeneic hematopoietic stem cell transplantation (ALLOHSCT) is a well-established therapy option for many people suffering from cancer and other disorders.The procedure involves extracting a fraction of a healthy donor’s bone marrow or stem cells and infused to the patient so that healthy donor’s stem cells replace the faulty blood stem cells of the patient to maintain the child’s toxicity profile to a minimum, a carefully crafted chemotherapy regimen was started. The child did have a few infectious episodes, but with the right treatment, he made a full recovery. After four cycles of chemotherapy, the blood cancer was in remission.
The BGS Gleneagles Global Hospital’s expert team, which included Dr. Govind Eriat, Consultant- Hematology & BMT Medicine, Dr. Rajeev Vijayakumar, Consultant, Medical Oncologist, Haemato-Oncologist & BMT Physician, and Dr. Prerana S. Nesargi, Associate Consultant, Paediatric Oncology and Haematology, achieved a major victory by curing the child of blood cancer. The next stage was HAPLO ALLO HSCT; else, the child would not survive for long either bone marrow failure or relapse of blood cancer.
“Appropriate transplant treatment was commenced, but it was discouraging because the child did not have a sibling and we were unable to identify a match in the donor registry. After a thorough workup and counseling of the very high-risk status of HSCT in terms of mortality and morbidity, one of the parent volunteered to be the donor as a desperate measure.A haploidentical HSCT transplant, often known as a partly matched or half-matched transplant, was performed in June. The child’s journey through hospitalization and discharge was like riding a roller coaster, with the child discharged from the hospital on full engraftment. This case serves as an illustration of how confidence in science, perseverance, and outstanding teamwork can produce miracles and inspire us to treat our patients” added Dr. Prerana S Nesargi
