A case of successful management with splenectomy of intractable ascites due to congenital dyserythropoietic anemia type Ⅱ-induced cirrhosis

The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital...

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Published in:World journal of gastroenterology : WJG 2006-02, Vol.12 (5), p.818-821
Main Authors: Vassiliadis, Themistoklis, Garipidou, Vassilia, Perifanis, Vassilios, Tziomalos, Konstantinos, Giouleme, Olga, Patsiaoura, Kalliopi, Avramidis, Michalis, Nikolaidis, Nikolaos, Vakalopoulou, Sofia, Tsitouridis, Ioannis, Antoniadis, Antonios, Semertzidis, Panagiotis, Kioumi, Anna, Premetis, Evangelos, Eugenidis, Nikolaos
Format: Article
Language:English
Subjects:
Anemia, Dyserythropoietic, Congenital - complications
Anemia, Dyserythropoietic, Congenital - pathology
Anemia, Dyserythropoietic, Congenital - surgery
Anemia, Hemolytic, Autoimmune - complications
Ascites - etiology
Ascites - surgery
Case Report
Female
Humans
Liver Cirrhosis - etiology
Liver Cirrhosis - pathology
Middle Aged
Splenectomy
硬化症状
脾切除术
贫血症
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Summary:The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II is the most frequent type. All types of congenital dyserythropoietic anemias distinctly share a high incidence of iron loading. Iron accumulation occurs even in untransfused patients and can result in heart failure and liver cirrhosis. We have reported about a patient who presented with liver cirrhosis and intractable ascites caused by congenital dyserythropoietic anemia type II. Her clinical course was further complicated by the development of autoimmune hemolytic anemia. Splenectomy was eventually performed which achieved complete resolution of ascites, increase of hemoglobin concentration and abrogation of transfusion requirements.
ISSN:1007-9327
2219-2840
DOI:10.3748/wjg.v12.i5.818