Diamond Blackfan anemia [DBA] in association with polydactyly: Response to antilymphocyte globulin[ALG]

ABSTRACT

An 8-month old Saudi Arabian boy presented with Diamond Blackfan Anaemia [DBA] and polydactyly. He required support with monthly red blood cell [RBC] transfusions. Although his bone marrow showed erythroblastopenia with giant pro-erythroblasts, examination of a later serum sample showed no evidence of B19 parvovirus DNA, and significant amounts of IgM and lg ti-B19 antibodies. He showed no clinical improvement on a 3-month course of prednisone alone, subsequently followed by concomitant oxymethalone for 5 months. However, a 9 day course of daily infusion of anti lymphocyte globin [ALG] was followed by a significant improvement in haemoglobin. Although he briefly suffered a relapse about 9 months later, he recovered spontaneously and has been in complete remission since. We conclude that some patients with DBA may benefit from treatment with ALG