Recently, a 3-year-old patient, Nashra Khan, was brought to Fortis Hospital Shalimar Bagh, where after a thorough medical evaluation she was diagnosed with hemophagocytic lymphohistiocutosis, a rarely occurring hereditary auto-immune condition with a five percent survival rate.
A multidisciplinary Approach of Treatment
The case was led by Dr Rahul Bhargava ( Director & Head, Blood Disorder, Hematology |Medical Oncology, Hematology & BMT) and Dr Amrita Chakrabarti (Associate Consultant, Hematology and Bone Marrow Transplant) who decided to follow a multi disciplinary approach for the treatment. On presentation, the patient had a fever, was suffering from diarrhea, and abdominal swelling. Her medical history indicated that her elder siblings had also reported similar conditions, before passing away. She was evaluated on an urgent basis – blood tests, scans, and a bone marrow test – which confirmed the diagnosis of familial HLH. She was carrying a very rare mutation in her genes, STXBP2, which (helped confirm this rare genetic disease called Familial HLH).
Dr Rahul Bhargava said, “We commenced her treatment with chemotherapy – an attempt to suppress her overactive, abnormal and self-harming immune system. We adopted the HLH protocol 94 and once the initiation phase was completed, we went ahead with a half-matched bone marrow transplant. The father donated his stem cells, which were collected, stored, and later transplanted into Nashra after treating her with Treosulphan, Fludarabine and TBI conditioning.”
Mr Mahipal Bhanot, Zonal Director, Fortis Hospital, Shalimar Bagh said, “Fortis Hospital Shalimar Bagh approaches every case in a manner which is beneficial for the patient. Our doctors remain committed to treating every patient even amid the pandemic and we are truly proud of them. This case was extremely challenging, but our doctors ensure that the best possible outcome was achieved. I congratulate them for their commitment to world class patient care.