This rare disorder has only 200 odd cases known worldwide with bone-marrow transplant data available for only 10-11 patients with variable outcomes
TIL Desk Lucknow/ Lucknow In a groundbreaking achievement, spearheaded by Dr. Priyanka Chauhan, a Hematologist & Bone Marrow Transplant Specialist, doctors at Apollomedics Hospital, Lucknow in Uttar Pradesh have successfully conducted the first allogenic stem cell transplant for Activated PI3K Delta Syndrome (APDS), a highly rare PRIMARY IMMUNODEFICIENCY DISORDER. The patient, a 14-year-old boy, had been diagnosed with the disorder at the age of seven. His condition required monthly immunoglobin infusions to replenish antibodies, which were not only expensive but had made the treatment availability a challenge for the humble family background of the patient. Last year, the patient and his family sought treatment at Apollo Medics Hospital in Lucknow. To their relief, the patient’s brother turned out to be a full HLA match donor for the bone marrow transplant.
“Primary immunodeficiency disorders are rare genetic conditions that pose significant challenges for patients and medical professionals alike due to recurrent or severe infections, autoimmune disorders, and increased susceptibility to cancer. This disorder, with only 200 reported cases worldwide, has seen limited success with bone marrow transplants, with only 10-11 cases conducted to date as per scientific literature. However, a breakthrough in Uttar Pradesh brought hope to a 14-year-old boy with the disorder, as he underwent a successful bone marrow transplant at Apollo Medics Hospital in Lucknow. With ongoing research and advancements in treatment options, there is hope for better outcomes and improved quality of life for those affected by this rare primary immunodeficiency disorder”, said Dr Priyanka Chauhan, Consultant, Haemotology and Oncology at Apollomedics Hospital, Lucknow.
The bone marrow transplant procedure was not without its challenges. The patient’s liver was involved in the disorder, further complicating the transplant process. Additionally, the patient had baseline lung disease due to repeated infections in past, that required careful management before and after the transplant. Respiratory infection was a major concern during transplant requiring antibiotic and antifungal treatment, also White blood cells (Granulocytes) were infused to boost the immune system and aid in the recovery process. Throughout the treatment, the patient received supportive care to manage symptoms and complications. Regular monitoring and follow-ups were essential to track progress and address any concerns promptly. Two months post-transplant,he has 98% functioning donor cells , he is in good health, signifying a positive outcome.
Dr. Mayank Somani while talking about groundbreaking achievement said, ”Primary Immunodeficiency poses significant challenges, and our achievement in conducting Uttar Pradesh’s first bone marrow transplant for this rare disorder is a beacon of hope. With only a handful of cases worldwide, our efforts and a bench of highly skilled doctors signified a crucial step in enhancing treatment options and fostering optimism for patients in our region.”
