How would you treat a patient with Wilson’s disease and neutropenia?

Professor of Medicine (Hematology), Active Emeritus at the Stanford School of Medicine in Stanford, California

This month, Stanley L. Schrier, MD, answers a question about a patient with Wilson’s disease and neutropenia.

CLINICAL DILEMMA

I was asked to see a 41-year-old male with Wilson’s disease. He has resulting cirrhosis and hypersplenism with a chronic pancytopenia. His leukopenia is worsening with an absolute neutrophil count of 400. His hemoglobin is mildly decreased by 2 g/dL and is currently at 8.3 g/dL, and his platelet count is stable at 73,000. He is continuing chronic zinc therapy for his Wilson’s disease. I am concerned that his zinc therapy has resulted in copper depletion as a cause for his worsened neutropenia, which more typically remains with an ANC of 1700. A vitamin B12 level and folic acid are normal, his LDH is normal, and his absolute reticulocyte count is 160 (6.4%). I am interested in further thoughts on evaluation and treatment.

EXPERTS MAKE THE CALL

This case is complicated by cirrhosis and hypersplenism. The patient is essentially pancytopenic, likely due to hypersplenism with an inadequate reticulocyte response of 160,000/µL. The role of zinc is to block copper absorption, but have you measured his recent copper – either urine or blood? Has his liver copper been adequately treated with penicillamine? Lastly, has the patient had a recent marrow to look for a fuller explanation of his pancytopenia, reticulocytopenia, or even ringed sideroblasts possibly caused by copper depletion.

How did readers respond? Check out You MADE the Call.

Disclaimer: ASH does not recommend or endorse any specific tests, physicians, products, procedures, or opinions, and disclaims any representation, warranty, or guaranty as to the same. Reliance on any information provided in this article is solely at your own risk.

SHARE