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

Abstract

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.

• Authors: Themistoklis Vassiliadis, Vassilia Garipidou, Vassilios Perifanis, Konstantinos Tziomalos, Olga Giouleme, Kalliopi Patsiaoura, Michalis Avramidis, Nikolaos Nikolaidis, Sofia Vakalopoulou, Ioannis Tsitouridis, Antonios Antoniadis, Panagiotis Semertzidis, Anna Kioumi, Evangelos Premetis, Nikolaos Eugenidis
• Journal: World journal of gastroenterology (World J Gastroenterol) Vol. 12 Issue 5 Pg. 818-21 (Feb 07 2006) ISSN: 1007-9327 [Print] United States
• PMID: 16521204 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Anemia, Dyserythropoietic, Congenital (complications, pathology, surgery)
• Anemia, Hemolytic, Autoimmune (complications)
• Ascites (etiology, surgery)
• Female
• Humans
• Liver Cirrhosis (etiology, pathology)
• Middle Aged
• Splenectomy