Acta Med. 2006, 49: 193-195

Successful Treatment of Iron Overload with Phlebotomies in Two Siblings with Congenital Dyserythropoietic Anemia – Type II (CDA-II)

Ladislav Chrobák

Charles University in Prague, Faculty of Medicine and University Hospital in Hradec Králové, 2nd Department of Medicine, Division of Clinical Hematology, Hradec Králové, Czech Republic

Received August 1, 2006
Accepted September 1, 2006


1. Brittenham GM, Griffith PM, Nienhuis AW et al. Efficacy of deferoxamine in preventing complications of iron overload in patiens with thalassemia major. N Engl J Med 1994; 331:567–73. <>
2. Ceci A, Baiardi P, Filisi M. The safety and effectiveness of deferiprone in a large – scale, 3-year study in Italian patiens. Br J Haematol 2002; 118: 330–6. <>
3. Chrobák L, Hůlek P, Nožička J. Kongenitální dyserythropoetická anémie – type II (CDA-II) u tří sourozenců s dlouhodobým sledováním a přetížením železem. Acta Medica (Hradec Králové) Suppl. 2004; 47 (1):29–33.
4. Chrobák L, Matysová J. Excess of Pappenheimer bodies (siderocytes) in two splenectomized sibling with congenital dyserythropoietic anemia – type II (CDAII) and iron overload. Acta Medica (Hradec Králové) 2004; 47(3):187–8.
5. Chrobák L, Špaček J. Příznivý vliv splenektomie na anémii u tří sourozenců s kongenitální dyserythropoietickou anémií – type II (HEMPAS) (ultrastrukturální změny erytrocytů po splenektomii). Vnitř Lék 1997; 43:635–8.
6. Chrobák L, Radochová D, Smetana K et al. Congenital dyserytrhropoietic anaemia, type II (HEMPAS) in three siblings. Folia Haematol (Leipzig) 1980; 107: 628–40.
7. Crookston JH, Crookston MC, Burnie KL et al. Hereditary erythroblastic multinuclearity associated with a positive acidified – serum test: a type of congerital dyserythropoietic anemia. Br J Haematol 1969; 17:11–26. <>
8. Fisher R, Tiemann CD, Engelhardt R et al. Assessment of iron stores in children with transfusion siderosis by biomagnetic liver susceptometry. Amer J Hematol 1999; 60:289–99. <<289::AID-AJH7>3.0.CO;2-W>
9. Heimpel H, Anselstetter V, Chrobák L et al. Congenital dyserythropoietic anemia type II: epidemiology, clinical appearance, and prognosis based on long-term observation. Blood 2003; 102:4576–81. <>
10. Hofmann WK, Kaltwasser JP, Hoelzer D et al. Successful treatment of iron overload by flebotomies in a patient with severe congenital dyserythropoietic anemia type II (letter). Blood 1997; 89:3068–9.
11. Piga A, Roggero S, Longo F: Pathogenesis and management of iron – loading anemias. Hematology (EHA Educ. Program) 2006; 2:42–6.
12. Van Steenbergen W, Matthijs G, Roskams T, Fevery J. Noniatrogenic haemochromatosis in congenital dyserythropoietic anaemia type II is not related to C282Y and A63D mutations in the HFE gene: report on two brothers. Acta Clin Belg 2002; 57:79–84. <>
13. Wickramasinghe SN. Response of CDA type I to alpha – interferon. European J Haematology 1997; 58:121–3. <>
14. Wickramasinghe SN, Wood WG. Advances in the understanding of the congenital dyserythropoietic anaemias. Br J Haematol 2005; 131:431–46. <>
front cover

ISSN 1211-4286 (Print) ISSN 1805-9694 (Online)

Open access journal