Wilson's disease and secondary copper hemochromatoses in hematological practice
Aim. To characterize clinical, diagnostic and therapeutic syndromes of copper overloading in patients with hepatic lesion in combination with hemopoietic disorder. Material and methods. Treatment results and diagnostic findings are presented for patients with clinical picture of liver cirrhosis, cyt...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | Russian |
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"Consilium Medicum" Publishing house
2004-07-01
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| Series: | Терапевтический архив |
| Subjects: | |
| Online Access: | https://ter-arkhiv.ru/0040-3660/article/view/29996 |
| _version_ | 1819005237899296768 |
|---|---|
| author | A I Vorobyev E A Lukina E P Sysoeva A A Levina T I Solovyeva |
| author_facet | A I Vorobyev E A Lukina E P Sysoeva A A Levina T I Solovyeva |
| author_sort | A I Vorobyev |
| collection | DOAJ |
| description | Aim. To characterize clinical, diagnostic and therapeutic syndromes of copper overloading in patients with hepatic lesion in combination with hemopoietic disorder.
Material and methods. Treatment results and diagnostic findings are presented for patients with clinical picture of liver cirrhosis, cytopenia and copper overloading. The examination included standard clinical and specific tests, morphological investigation of the bone marrow, copper metabolism in dynamics.
Results. A case of a patient is reported in whom Wilson's disease presented in debut with a picture of decompensated liver cirrhosis and immune thrombocytopania complicated by recurrent hemorrhagic syndrome. D-penicillomine treatment initiated ex juvantibus allowed verification of the diagnosis of Wilson's disease and achievement of marked clinical response.
In another case laboratory signs of copper overloading were revealed in a patient with liver cirrhosis of viral etiology (HBsAg+) and deep cytopenia associated with unejfective hemopoiesis. Chelator therapy with D-penicillamine regressed cytopenic syndrome and improved functional capacity of the liver. Conclusion. Primary or secondary nature of copper overloading in patients with hepatic cirrhosis and critical cytopenia, pathogenesis of cytopenic syndrome, practical significance of copper hemochromatoses diagnosis are discussed. |
| first_indexed | 2024-12-20T23:49:36Z |
| format | Article |
| id | doaj.art-a4d0ee19f3fb45138cef67f7c39797c1 |
| institution | Directory Open Access Journal |
| issn | 0040-3660 2309-5342 |
| language | Russian |
| last_indexed | 2024-12-20T23:49:36Z |
| publishDate | 2004-07-01 |
| publisher | "Consilium Medicum" Publishing house |
| record_format | Article |
| series | Терапевтический архив |
| spelling | doaj.art-a4d0ee19f3fb45138cef67f7c39797c12022-12-21T19:22:52Zrus"Consilium Medicum" Publishing houseТерапевтический архив0040-36602309-53422004-07-01797555927024Wilson's disease and secondary copper hemochromatoses in hematological practiceA I VorobyevE A LukinaE P SysoevaA A LevinaT I SolovyevaAim. To characterize clinical, diagnostic and therapeutic syndromes of copper overloading in patients with hepatic lesion in combination with hemopoietic disorder. Material and methods. Treatment results and diagnostic findings are presented for patients with clinical picture of liver cirrhosis, cytopenia and copper overloading. The examination included standard clinical and specific tests, morphological investigation of the bone marrow, copper metabolism in dynamics. Results. A case of a patient is reported in whom Wilson's disease presented in debut with a picture of decompensated liver cirrhosis and immune thrombocytopania complicated by recurrent hemorrhagic syndrome. D-penicillomine treatment initiated ex juvantibus allowed verification of the diagnosis of Wilson's disease and achievement of marked clinical response. In another case laboratory signs of copper overloading were revealed in a patient with liver cirrhosis of viral etiology (HBsAg+) and deep cytopenia associated with unejfective hemopoiesis. Chelator therapy with D-penicillamine regressed cytopenic syndrome and improved functional capacity of the liver. Conclusion. Primary or secondary nature of copper overloading in patients with hepatic cirrhosis and critical cytopenia, pathogenesis of cytopenic syndrome, practical significance of copper hemochromatoses diagnosis are discussed.https://ter-arkhiv.ru/0040-3660/article/view/29996wilson's diseasecopper overloadingcytopeniahepatic cirrhosisd-penicillamine |
| spellingShingle | A I Vorobyev E A Lukina E P Sysoeva A A Levina T I Solovyeva Wilson's disease and secondary copper hemochromatoses in hematological practice Терапевтический архив wilson's disease copper overloading cytopenia hepatic cirrhosis d-penicillamine |
| title | Wilson's disease and secondary copper hemochromatoses in hematological practice |
| title_full | Wilson's disease and secondary copper hemochromatoses in hematological practice |
| title_fullStr | Wilson's disease and secondary copper hemochromatoses in hematological practice |
| title_full_unstemmed | Wilson's disease and secondary copper hemochromatoses in hematological practice |
| title_short | Wilson's disease and secondary copper hemochromatoses in hematological practice |
| title_sort | wilson s disease and secondary copper hemochromatoses in hematological practice |
| topic | wilson's disease copper overloading cytopenia hepatic cirrhosis d-penicillamine |
| url | https://ter-arkhiv.ru/0040-3660/article/view/29996 |
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