Red cell aplasia following prolonged D-penicillamine therapy.

Gollan, John L.; Hussein, S.; Av, Hoffbrand; Sherlock, Sheila

doi:10.1136/jcp.29.2.135

Cited by 16 publications

(4 citation statements)

References 20 publications

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“…Our patient presented with anemia and neutropenia due to hypocupremia following high-dose zinc monotherapy for 4 years and 4 months, and her hematological parameters recovered after zinc withdrawal for 4 months. Some physicians may suggest switching to D-penicillamine or trientine, as de-coppering drug, both of them can also lead to hypocupremia-related anemia, as previously reported [25, 26]. The details regarding zinc therapy and anemia in these 9 patients are listed in Table 2.…”

Section: Discussionmentioning

confidence: 82%

Anemia following zinc treatment for Wilson’s disease: a case report and literature review

et al. 2019

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Background Zinc therapy is considered an effective and safe treatment for Wilson’s disease. Hypocupremia-related anemia is rarely reported after long-term zinc administration or combination therapy with copper-chelating agent. Case presentation We herein report a 12-year-old girl with pre-symptomatic Wilson’s disease diagnosed 5 years ago who presented with severe anemia after high-dose oral zinc for 4 years and 4 months. Her hemoglobin was gradually restored to the normal range after the adjustment of zinc dose and diet therapy for 4 months. A review of the literature revealed eight patients with hypocupremia-associated anemia following zinc therapy for Wilson’s disease, including 7 adults and 1 child. The only child patient was a 16-year-old boy, in whom the zinc therapy was succession to penicillamine administration. Conclusions This is the first report worldwide that a child developed severe anemia following high-dose single zinc administration for Wilson’s disease. It highlights the importance of regular follow-up during zinc treatment and the involvement of specialists in the long-term management of Wilson’s disease. We hope that this will alert pediatricians the issue of zinc over-treatment.

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Section: Discussionmentioning

confidence: 82%

Anemia following zinc treatment for Wilson’s disease: a case report and literature review

et al. 2019

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“…Agranulocytosis, though rare, is more common in the first year of therapy and is usually reversible on stopping the drug (Golding, Wilson and Day, 1970). Selective red cell aplasia has occurred after prolonged treatment of a patient with Wilson's disease with Dpenicillamine (Gollan et al, 1976). The occurrence of erythro-myeloid aplasia with preservation of platelet production confirms that D-penicillamineinduced blood dyscrasias can selectively involve any of the haemopoietic cell lines.…”

Section: Discussionmentioning

confidence: 91%

Erythro-myeloid aplasia following D-penicillamine treatment in primary biliary cirrhosis

Jones¹,

Corringham²,

Jain³

1978

Postgraduate Medical Journal

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Summary A woman with primary biliary cirrhosis and a high liver copper concentration was treated with D-penicillamine as a participant in a controlled trial of the drug. After 14 weeks she developed a Pseudomonas septicaemia and was found to have erythro-myeloid aplasia. Similar, but not identical blood dyscrasias have previously been reported as complications of D-penicillamine therapy. She survived after treatment with antibiotics and granulocyte transfusions, and bone marrow function returned to normal.

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“…Bone marrow is often involved in WD afflicted subjects, either as a part of copper overload, copper-chelator penicillamine related or subsequent to over-chelation. It presents by cytopenia, marrow trilineage dysplasia (11) and bone marrow aplasia (12).…”

Section: Introductionmentioning

confidence: 99%

Structural and Functional Cardiac Changes in Children with Wilson Disease

Kotb

Mostafa

Badr

et al. 2022

Pediatric Sciences Journal

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Background: Wilson disease (WD) is an autosomal recessive disease with copper overload. Its clinical picture depends on specific tissue/system damage by the excess copper. Aim of the work:We aimed to study prospectively the phenotypic spectrum of structural and functional cardiac changes among children with WD. Methods: 16 children with confirmed WD underwent electrocardiography (ECG), conventional and tissue Doppler echocardiography. Results: ECG was normal in 11 patients (68.7%), inverted T was detected in 2 (12.5%), ST elevation in 2 (12.5%) while P-pulmonale and inverted T were detected in 1 (6.25%). Five patients (31.25%) had mild and one (6.25%) had severe tricuspid regurgitation. Two girls (12.5%) with WD had underlying congenital heart defects, one had atrial septal defect (ASD) and another had double inlet left ventricle (DILV), malposed great vessels and severe pulmonary stenosis. There was a positive correlation between LV mass and duration of treatment (r=0.559, p=0.030), and a negative correlation between age of onset and LV mass index (r=0.600, p=0.018). There was no significant correlation between age of onset and duration of treatment with myocardial perfusion imaging (MPI) or tissue Doppler parameters. Conclusion: WD in children is associated with cardiac structural and functional changes including congenital structural heart malformations; ASD and DILV. Future research is needed to verify if ASD and DILV in WD are embryonic presentations of copper overload in WD.

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Red cell aplasia following prolonged D-penicillamine therapy.

Cited by 16 publications

References 20 publications

Anemia following zinc treatment for Wilson’s disease: a case report and literature review

Anemia following zinc treatment for Wilson’s disease: a case report and literature review

Erythro-myeloid aplasia following D-penicillamine treatment in primary biliary cirrhosis

Structural and Functional Cardiac Changes in Children with Wilson Disease

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