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

Several forms of hereditary anemia with ineffective erythropoiesis, more benign than thalassemia. 3 types are based upon bone marrow & serologic findings: 1) CDA-1 a) Megaloblastic changes with some binuclearity, b) internuclear chromatin bridges c) macrocytic anemia d) autosomal recessive 2) CDA-2 a) binuclearity & multinuclearity of erythrocyte precursors b) pluripolar mitosis c) karyorrhexis d) normocytic anemia e) positive acidified serum test (HEM-PAS) f) RBC have antigen not present on normal or PNH cells, only 1/3 of normal sera have antibody to the antigen g) autosomal recessive 3) CDA-3 a) giant erythroid precursors b) pronounced multinuclearity c) autosomal dominant

Specific

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

General

anemia congenital anomaly (birth defect)

References

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