Literature DB >> 25332639

Cytopenia and Bone Marrow Dysplasia in a Case of Wilson's Disease.

Aarathi R Rau1, M Usha1, Pooja Mallya2, A T K Rau2.   

Abstract

We describe a sixteen year old with Wilson's disease on copper chelation and subsequent high dose oral zinc who developed severe anemia and neutropenia. Bone marrow aspirate done to evaluate the cause of bicytopenia revealed trilineage dysplasia. Correlating the clinical context with bone marrow and biochemical parameters, copper deficiency was suspected and he was given a trial of therapy, following which the hematological parameters improved. This case highlights hypocupremia as a reversible cause of bone marrow dysplasia in patients with Wilson's disease on chelation, where serum copper levels are not useful in the diagnosis. We also believe that monitoring of the blood counts in patients on copper chelation may provide a clue to impending copper deficiency.

Entities:  

Keywords:  Bone marrow dysplasia; Cytopenia; Hypocupremia; Wilson’s disease

Year:  2014        PMID: 25332639      PMCID: PMC4192249          DOI: 10.1007/s12288-014-0456-3

Source DB:  PubMed          Journal:  Indian J Hematol Blood Transfus        ISSN: 0971-4502            Impact factor:   0.900


  9 in total

1.  Excessive zinc ingestion. A reversible cause of sideroblastic anemia and bone marrow depression.

Authors:  E R Broun; A Greist; G Tricot; R Hoffman
Journal:  JAMA       Date:  1990-09-19       Impact factor: 56.272

2.  Cellular copper content modulates differentiation and self-renewal in cultures of cord blood-derived CD34+ cells.

Authors:  Tony Peled; Efrat Landau; Eugenia Prus; Abraham J Treves; Arnon Nagler; Eitan Fibach
Journal:  Br J Haematol       Date:  2002-03       Impact factor: 6.998

3.  Zinc-induced copper deficiency: a report of three cases initially recognized on bone marrow examination.

Authors:  Monte S Willis; Sara A Monaghan; Michael L Miller; Robert W McKenna; Wiley D Perkins; Barry S Levinson; Vikas Bhushan; Steven H Kroft
Journal:  Am J Clin Pathol       Date:  2005-01       Impact factor: 2.493

Review 4.  Update on anemia and neutropenia in copper deficiency.

Authors:  John Lazarchick
Journal:  Curr Opin Hematol       Date:  2012-01       Impact factor: 3.284

Review 5.  Hypocupremia associated cytopenia and myelopathy: a national retrospective review.

Authors:  Alemayehu A Gabreyes; Hina Naz Abbasi; Kirsten P Forbes; Grant McQuaker; Andrew Duncan; Ian Morrison
Journal:  Eur J Haematol       Date:  2012-11-22       Impact factor: 2.997

6.  Copper deficiency masquerading as myelodysplastic syndrome.

Authors:  Xylina T Gregg; Vishnu Reddy; Josef T Prchal
Journal:  Blood       Date:  2002-08-15       Impact factor: 22.113

7.  Element of caution: a case of reversible cytopenias associated with excessive zinc supplementation.

Authors:  Julie A Irving; Andre Mattman; Gillian Lockitch; Kevin Farrell; Louis D Wadsworth
Journal:  CMAJ       Date:  2003-07-22       Impact factor: 8.262

8.  Acquired sideroblastic anaemia during treatment of Wilson's disease with triethylene tetramine dihydrochloride.

Authors:  L Condamine; O Hermine; P Alvin; M Levine; C Rey; V Courtecuisse
Journal:  Br J Haematol       Date:  1993-01       Impact factor: 6.998

9.  Assignment of the gene for Wilson disease to chromosome 13: linkage to the esterase D locus.

Authors:  M Frydman; B Bonné-Tamir; L A Farrer; P M Conneally; A Magazanik; S Ashbel; Z Goldwitch
Journal:  Proc Natl Acad Sci U S A       Date:  1985-03       Impact factor: 11.205

  9 in total
  1 in total

1.  A rare case of Wilson disease associated with intracerebral hemorrhage.

Authors:  Shalendra Singh; Venigalla Sri Krishna; Nipun Gupta; Priya Taank; Vikas Marwah
Journal:  Korean J Anesthesiol       Date:  2020-05-20
  1 in total

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