Circulating nucleated red blood cells following splenectomy in a patient with congenital dyserythropoietic anemia.

A 24-year-old white female with a 10-year history of juvenile rheumatoid arthritis and splenomegaly developed numerous circulating nucleated red blood cells (NRBC) following splenectomy for chronic abdominal pain. Subsequent evaluation revealed the presence of a congenital dyserythropoietic anemia (CDA) with atypical features. Circulating NRBCs have been reported following splenectomy in three other cases of CDA, each of which had atypical features and did not fit into the customary classification of types I-IV. Follow-up of our patient at 4 years revealed no untoward consequences of persistent NRBCs in her circulation.