title: 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.affiliation: 2nd Propedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration General Hospital, Thessaloniki, Greece.
authors.forename: Themistoklis
authors.initials: T
authors.lastname: Vassiliadis
authors.forename: Vassilia
authors.initials: V
authors.lastname: Garipidou
authors.forename: Vassilios
authors.initials: V
authors.lastname: Perifanis
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Tziomalos
authors.forename: Olga
authors.initials: O
authors.lastname: Giouleme
authors.forename: Kalliopi
authors.initials: K
authors.lastname: Patsiaoura
authors.forename: Michalis
authors.initials: M
authors.lastname: Avramidis
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Nikolaidis
authors.forename: Sofia
authors.initials: S
authors.lastname: Vakalopoulou
authors.forename: Ioannis
authors.initials: I
authors.lastname: Tsitouridis
authors.forename: Antonios
authors.initials: A
authors.lastname: Antoniadis
authors.forename: Panagiotis
authors.initials: P
authors.lastname: Semertzidis
authors.forename: Anna
authors.initials: A
authors.lastname: Kioumi
authors.forename: Evangelos
authors.initials: E
authors.lastname: Premetis
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Eugenidis
mesh_terms: D000742:Anemia, Dyserythropoietic, Congenital / Q000150:complications / Q000473:pathology / Q000601:surgery*; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications; D001201:Ascites / Q000209:etiology / Q000601:surgery; D005260:Female; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology / Q000473:pathology; D008875:Middle Aged; D013156:Splenectomy