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

World Journal of Gastroenterology : WJG
Themistoklis VassiliadisNikolaos Eugenidis

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.

References

Nov 1, 1992·American Journal of Clinical Pathology·T C GreinerI Mahmood
May 1, 1985·Archives of Surgery·W W Coon
Aug 1, 1983·British Journal of Haematology·M CazzolaE Ascari
Mar 1, 1994·World Journal of Surgery·J ElcherothD Franco
Dec 31, 1998·Gastroenterology·B R BaconJ H Hoofnagle
Jun 26, 1999·Annals of Internal Medicine·B R BaconR K Wolff
Oct 18, 2001·Current Opinion in Hematology·L D Petz
Oct 18, 2001·Seminars in Hematology·B Wonke
Jun 26, 2002·Journal of the American College of Surgeons·Marshall J OrloffSusan L Orloff
Feb 20, 2003·Journal of Hepatology·Jaime BoschRoberto Groszmann
Sep 23, 2003·European Journal of Gastroenterology & Hepatology·Johanna A Kremer HovingaJean-François Dufour
May 1, 1997·Clinics in Liver Disease·D A Iannitti, J M Henderson
Jan 22, 2005·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Thomas D BoyerUNKNOWN American Association for the Study of Liver Diseases

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