Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2006 Feb 7;12(5):818-21.
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  1 , 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
Affiliations
Review

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

Themistoklis Vassiliadis et al. World J Gastroenterol. .

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.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Peripheral blood smear showing binucleated normoblasts.
Figure 2
Figure 2
Liver biopsy showing marked siderosis of hepatocytes (curved arrows), Kupffer cells (arrowheads with ball) and portal macrophages (arrows) (Perl’s stain, ×200).
See this image and copyright information in PMC

References

    1. Heimpel H. Congenital dyserythropoietic anemias: epidemiology, clinical significance, and progress in understanding their pathogenesis. Ann Hematol. 2004;83:613–621. - PubMed
    1. Cazzola M, Barosi G, Bergamaschi G, Dezza L, Palestra P, Polino G, Ramella S, Spriano P, Ascari E. Iron loading in congenital dyserythropoietic anaemias and congenital sideroblastic anaemias. Br J Haematol. 1983;54:649–654. - PubMed
    1. Iolascon A, D'Agostaro G, Perrotta S, Izzo P, Tavano R, Miraglia del Giudice B. Congenital dyserythropoietic anemia type II: molecular basis and clinical aspects. Haematologica. 1996;81:543–559. - PubMed
    1. Heimpel H, Anselstetter V, Chrobak L, Denecke J, Einsiedler B, Gallmeier K, Griesshammer A, Marquardt T, Janka-Schaub G, Kron M, et al. Congenital dyserythropoietic anemia type II: epidemiology, clinical appearance, and prognosis based on long-term observation. Blood. 2003;102:4576–4581. - PubMed
    1. Greiner TC, Burns CP, Dick FR, Henry KM, Mahmood I. Congenital dyserythropoietic anemia type II diagnosed in a 69-year-old patient with iron overload. Am J Clin Pathol. 1992;98:522–525. - PubMed

MeSH terms