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

CDA-2 is one of several forms of hereditary anemia with ineffective erythropoiesis. It is the most common form. Inheritance is autosomal recessive. 1) binuclearity & multinuclearity of erythrocyte precursors 2) pluripolar mitosis 3) karyorrhexis 4) normocytic anemia 5) positive acidified serum test (HEM-PAS) 6) RBC have antigen not present on normal or PNH cells, only 1/3 of normal sera have antibody to the antigen

References

Clinical Diagnosis & Management by Laboratory Methods, 19th edition, J.B. Henry (ed), W.B. Saunders Co., Philadelphia, PA. 1996, pg 631