Background:

Wilson’s disease is an autosomal recessive disorder of copper metabolism, typically treated with D-penicillamine. While effective, D-penicillamine has been associated with hematologic complications. Disseminated intravascular coagulation (DIC) is a rare but life-threatening coagulopathy that can arise in this context.

Case Description:

We present a 13-year-old male with Wilson’s disease who initially presented with progressive edema, jaundice, and neuropsychiatric symptoms. Workup revealed advanced hepatic dysfunction (Child-Pugh Class C) confirmed by laboratory, imaging, and histopathological findings. The patient was stabilized and discharged on D-penicillamine and zinc therapy pending liver transplantation. Two weeks later, he was readmitted with acute gastrointestinal bleeding, hypotension, and laboratory evidence of DIC. Despite aggressive resuscitative efforts, his condition rapidly deteriorated, culminating in multi-organ failure and death.

Wilson’s disease-related hepatic dysfunction creates a procoagulant environment. D-penicillamine, although a cornerstone in treatment, has been implicated in rare cases of DIC. The temporal association between D-penicillamine initiation and the onset of DIC in this patient suggests a potential causal link, supported by previous reports. The mechanisms may involve immune-mediated endothelial injury or exacerbation of hepatic dysfunction, emphasizing the need for heightened vigilance in patients with pre-existing coagulopathies.

This case highlights the potential for D-penicillamine to precipitate DIC in patients with Wilson’s disease and advanced liver dysfunction. Clinicians should carefully assess coagulation status before initiating therapy and consider alternative chelators, such as trientine, in high-risk patients. Early recognition and prompt management are essential to mitigate life-threatening complications.

This case highlights the potential for D-penicillamine to precipitate DIC in patients with Wilson’s disease and advanced liver dysfunction. Clinicians should carefully assess coagulation status before initiating therapy and consider alternative chelators, such as trientine, in high-risk patients. Early recognition and prompt management are essential to mitigate life-threatening complications.