Diamond-Blackfan anemia (DBA) is a rare congenital hypoplastic anemia<sup>32 Diamond L.K., Blackfan K.D. Hypoplastic anemia Am J Dis Child 1938 ;  56 : 464

Cliccare qui per andare alla sezione Riferimenti, 66 Josephs H.W. Anaemia of infancy and early childhood Medicine 1936 ;  15 : 307  [cross-ref]

Cliccare qui per andare alla sezione Riferimenti</sup> that usually presents early in infancy. Retrospective studies in the United Kingdom and the Netherlands are consistent with an incidence of 4–5 per million live births,<sup>12 Ball S.E., McGuckin C.P., Jenkins G. , e al. Diamond-Blackfan anaemia in the UK: Analysis of 80 cases from a 20-year birth cohort Br J Haematol 1996 ;  94 : 645

Cliccare qui per andare alla sezione Riferimenti, 24 Bresters D., Bruin M.C.A., van Dijken P.J. Congenitale hypoplastische anemie in Nederland (1963–1989) Tijdschrift voor Kindergeneeskunde 1991 ;  59 : 203

Cliccare qui per andare alla sezione Riferimenti</sup> with evidence of inheritance (dominant and recessive) in perhaps 12% to 25% of cases.<sup>3 Alter B.P., Young N.S. The bone marrow failure syndromes Hematology of Infancy and Childhood Philadelphia: WB Saunders (1993).  216

Cliccare qui per andare alla sezione Riferimenti, 48 Glader B.E. Diagnosis and management of red cell aplasia in children Hematol Oncol Clin North Am 1987 ;  1 : 431

Cliccare qui per andare alla sezione Riferimenti, 126 Vlachos A., Lipton J.M. Bone marrow failure in children Curr Opin Pediatr 1996 ;  8 : 33

Cliccare qui per andare alla sezione Riferimenti</sup> Congenital anomalies, in particular of the head and upper limbs, are present in about a quarter of reported patients. The disease is characterized by a moderate-to-severe macrocytic anemia, occasional neutropenia and/or thrombocytosis, and a normocellular bone marrow with erythroid hypoplasia. Although proerythroblasts may be present or even increased, normoblasts are usually markedly diminished or absent. The majority of patients (75%) respond to prednisone, and erythropoiesis can then be maintained in some cases with almost homeopathic doses of the drug. Nonresponders are usually transfusion-dependent, although responses to androgen have been observed.<sup>33 Diamond L.K., Wang W.C., Alter B.P. Congenital hypoplastic anemia Adv Pediatr 1976 ;  22 : 349

Cliccare qui per andare alla sezione Riferimenti, 58 Hardisty R.M. Diamond-Blackfan anaemia Congenital Disorders of Erythropoiesis: Ciba Foundation Symposium 37 (New Series) Amsterdam: Elsevier/Excerpta Medica/North Holland (1976).  89

Cliccare qui per andare alla sezione Riferimenti, 73 Lipton J.M., Nathan D.G. Aplastic and hypoplastic anemia Pediatr Clin North Am 1980 ;  27 : 217

Cliccare qui per andare alla sezione Riferimenti, 110 Sieff C.A. Pure red cell aplasia Br J Haematol 1983 ;  54 : 331

Cliccare qui per andare alla sezione Riferimenti</sup>