congenital dyserythropoietic anemia (CDA-II or HEM-PAS)

Introduction

Several forms of hereditary anemia with ineffective erythropoiesis, more benign than thalassemia.

3 types are based upon bone marrow & serologic findings:

• CDA-1
  • Megaloblastic changes with some binuclearity,
  • internuclear chromatin bridges
  • macrocytic anemia
  • autosomal recessive
• CDA-2
  • binuclearity & multinuclearity of erythrocyte precursors
  • pluripolar mitosis
  • karyorrhexis
  • normocytic anemia
  • positive acidified serum test (HEM-PAS)
  • RBC have antigen not present on normal or PNH cells, only 1/3 of normal sera have antibody to the antigen
  • autosomal recessive
• CDA-3
  • giant erythroid precursors
  • pronounced multinuclearity
  • autosomal dominant

More general terms

• anemia
• congenital anomaly (birth defect)

More specific terms

• congenital dyserythropoietic anemia (CDA-1)
• congenital dyserythropoietic anemia (CDA-2 or HEM-PAS)
• congenital dyserythropoietic anemia (CDA-3)