First in Telugu states, baby with Griscelli Syndrome undergoes bone marrow transplant

Griscelli Syndrome is an inherited condition characterised by unusually light skin and light silvery-gray hair starting in infancy.

BySumit Jha

Published Oct 09, 2022 | 2:37 PMUpdatedOct 11, 2022 | 4:44 PM

Griscelli Syndrome

A 12-month-old baby with Griscelli Syndrome (GS) became one of the youngest in the Telugu-speaking states to successfully undergo a Bone Marrow Transplant (BMT) at the American Oncology Institute (AOI) in Hyderabad.

Symptoms of GS are unusually light (hypopigmented) skin and light silvery-grey hair, which start to be exhibited in the patient in infancy.

GS usually presents in infancy or early childhood, in most cases between the ages of four months and seven years. It has been successfully treated with stem-cell transplantation.

Griscelli Syndrome is rare, with a prevalence of less than one per million. According to the AOI, only 150 cases of it have been reported worldwide, of which 10 cases are from India.

The baby with Griscelli Syndrome was suffering from a recurrent infection due to immune system abnormalities, in addition to having hypopigmented skin and a silvery-grey sheen of hair.

Fortunately, the kid’s four-year-old elder sibling was the perfect match. However, due to the patient’s young age, the entire procedure was complicated.

Explaining the patient’s serious condition, Dr CS Ranjith Kumar, a consultant paediatric hemato-oncologist at AOI, said: “Transplant-related mortality remains the major challenge in children with primary immune deficiency disorders undergoing BMT. The treating paediatrician should detect these symptoms early for successful outcomes.”

He added: “India is making fast strides in BMT for PID (paediatric immunodeficiency), and in the future, we should be able to diagnose and transplant early and save more lives with better quality of life.”

The AOI has performed more than 70 BMT surgeries in the last two years, of which at least 25 were on paediatric patients.

Dr Prabhakar P, Regional Chief Operating Officer of AOI, said, “The number of patients seeking BMT in India has increased over the last five years. Highly experienced paediatric hemato-oncologists are needed to improve outcomes.”