Vassiliadis T, Garipidou V, Perifanis V, Tziomalos K, Giouleme O, Patsiaoura K, Avramidis M, Nikolaidis N, Vakalopoulou S, Tsitouridis I, Antoniadis A, Semertzidis P, Kioumi A, Premetis E, Eugenidis N. A case of successful management with splenectomy of intractable ascites due to congenital dyserythropoietic anemia type II-induced cirrhosis. World J Gastroenterol 2006; 12(5): 818-821 [PMID: 16521204 DOI: 10.3748/wjg.v12.i5.818]
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World J Gastroenterol. Feb 7, 2006; 12(5): 818-821 Published online Feb 7, 2006. doi: 10.3748/wjg.v12.i5.818
A case of successful management with splenectomy of intractable ascites due to congenital dyserythropoietic anemia type II-induced cirrhosis
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
Themistoklis Vassiliadis, Vassilia Garipidou, Vassilios Perifanis, Konstantinos Tziomalos, Olga Giouleme, Michalis Avramidis, Nikolaos Nikolaidis, Sofia Vakalopoulou, Panagiotis Semertzidis, Nikolaos Eugenidis, 2nd Propedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration General Hospital, Thessaloniki, Greece
Kalliopi Patsiaoura, Department of Pathology, Hippokration General Hospital, Thessaloniki, Greece
Ioannis Tsitouridis, Department of Radiology, Papageorgiou General Hospital, Thessaloniki, Greece
Antonios Antoniadis, Organ Transplant Unit, Aristotle University of Thessaloniki, Hippokration General Hospital, Thessaloniki, Greece
Anna Kioumi, Department of Hematology, Papageorgiou General Hospital, Thessaloniki, Greece
Received: June 22, 2005 Revised: June 29, 2005 Accepted: July 28, 2005 Published online: February 7, 2006
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.
