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

Here’s how readers responded to a You Make the Call question about a patient with Wilson’s disease and neutropenia?


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I would start with a marrow biopsy and peripheral blood film. If the MCV was elevated, if red cells were dimorphic, or if Pappenheimer bodies or marrow dysplasia were present, I’d start by reducing the zinc therapy. Also, I would measure copper levels, but would need to consult with an expert to interpret the levels in the context of Wilson’s disease.

Ian Morison, MD
Haematologist/Clinical Pathologist
Southern Community Laboratories
New Zealand

It would be helpful to know what specific drug the patient is being treated with for his Wilson’s disease. I suspect he is receiving penicillamine, and that this has caused drug-induced lupus and that his pancytopenia is a result of this autoimmune problem. I would switch him to a different drug for copper chelation and possibly treat with immune suppression to reduce the autoimmune issues.

Brian R. Curtis, PhD, D(ABMLI)
Director, Platelet & Neutrophil
Immunology Lab
Investigator, Blood Research Institute
BloodCenter of Wisconsin
Milwaukee, WI

I would advise to perform splenectomy as the patient has hypersplenism, and zinc does not cause neutropenia.

Dalia Nafea, MD
Assistant Lecturer of Hematology
Faculty of Medicine
Alexandria University
Alexandria, Egypt

If the patient is on penicillamine, it is possible an agranulocytosis picture. If not, I would put him on folic acid therapy, because a healthy liver is his major reservoir and and blood levels may not reflect its depletion.

Francisco Cuèllar – Ambrosi
Hematology
Clinica Leon XIII
Medellin

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