March 20 (Reuters) - Orchard Therapeutics, a unit of

Japan's Kyowa Kirin ( KYKOF ), priced its newly approved gene

therapy for a rare, life-threatening genetic disease on

Wednesday at $4.25 million, making it the most expensive drug in

the United States.

The therapy was approved on Monday, in a first for children

with a rare disorder called metachromatic leukodystrophy (MLD),

which causes damage to the nervous system and is estimated to

affect one in every 40,000 individuals in the United States.

Roughly half the patients with a late infantile form of the

disease do not live beyond five years from its onset.

The list price reflects "the value the therapy may

deliver to eligible patients and their families, as well the

potential long-term impact treatment may have on overall

healthcare utilization," the company said in a statement.

The list price is not necessarily what a patient pays, and

the final cost depends on insurance.

Orchard said the Institute for Clinical and Economic Review,

an independent drug pricing watchdog, had determined a

health-benefit price benchmark for its treatment at up to $3.94

million.

Australian drugmaker CSL's hemophilia B gene

therapy that was priced at $3.5 million in 2022 was the most

expensive drug at the time.

Orchard said it was working with commercial and government

insurers and other payers on outcome and value-based agreements

for coverage of the therapy.

(Reporting by Sneha S K and Manas Mishra in Bengaluru; Editing

by Shinjini Ganguli)