congenital dyserythropoietic anemia (CDA-2 or HEM-PAS)

Introduction

CDA-2 is one of several forms of hereditary anemia with ineffective erythropoiesis. It is the most common form. Inheritance is autosomal recessive.

• 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

More general terms

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

Additional terms

• acidified serum test (Ham test, acid hemolysin test)