A γ/δ T-cell receptor prolymphocytic leukemia and CD4-/CD8- double-negative immunophenotype in a pediatric patient. Academic Article uri icon

abstract

  • Acute idiopathic (immune) thrombocytopenic purpura (ITP) in the pediatric population is a disease in which autoimmune features are mainly self-limited, with a reported mortality of 0.1-0.5%. Major treatment requires intravenous gammaglobulins (i.v. IgG) and corticosteroids. Recently a new globulin, anti-D, has been introduced. The authors have treated 25 children suffering from acute idiopathic thrombocytopenic purpura, with an i.v. anti-D dose of 75 microg/kg as the first treatment. Eligibility criteria included a platelet count < 15,000 and Rh+. Post-treatment response was 76% > 20,000 platelets at 6-10 h and 80% > 50,000 platelets at 48 h; three patients developed chronic idiopathic thrombocytopenic purpura. There were 5/25 patients who did not respond to the initial dose and received i.v. IgG and corticosteroids, 2/5 with a positive response (platelets > 20,000). Side effects consisted of chills (9/25), fever > 38 degrees C (6/25), headache and vomiting (1/25), hemolysis (20/25) from 0.9-6.9 g%, and decrease in hemoglobin levels. One patient needed a blood transfusion after his Hbg decreased from 12.4 to 5.5 g%. The results indicate that anti-D is an effective treatment in acute ITP, but with side effects. Administration of steroids and antipyretics prior to anti-D treatment may prevent the side effects.

publication date

  • January 1, 2015