Copper-deficiency anemia and neutropenia during zinc maintenance therapy for Wilson disease associated with persistent strict dietary copper restriction.
Takako Nomura, Kiyoyuki Kobayashi, Maiko Murakami et al.
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Case in brief
A woman in her 60s with Wilson disease, hepatic and neurological involvement, and cirrhosis developed recurrent leukopenia and neutropenia three years after starting zinc maintenance therapy, followed by macrocytosis and rapidly worsening anemia. Her neutrophil count was 378/µL, mean corpuscular volume 120.9 fL, and hemoglobin 4.4 g/dL. Dietary history revealed persistent strict copper restriction, including long-term avoidance of copper-rich foods. After this restriction was stopped, she received red blood cell transfusion, and zinc acetate was reduced from 150 to 100 mg/day. Hemoglobin stabilized at approximately 10 g/dL, neutropenia improved, and macrocytosis resolved.
What made this case unusual
The cytopenias occurred during zinc maintenance therapy in the setting of persistent strict dietary copper restriction, a potentially overlooked contributor to copper deficiency.
Diagnostic / clinical pearl
In a patient with Wilson disease receiving zinc who develops cytopenias, reassess dietary copper restriction rather than attributing the findings solely to hypersplenism.