Intravenous gamma globulin for pure red cell aplasia – pro
Pure red cell aplasia (PRCA) describes a condition in which RBC precursors in bone marrow are nearly absent, while megakaryocytes and WBC precursors are usually present at normal levels. In 1922, Kaznelson recognized that this condition was a different entity than aplastic anemia. Pure red cell aplasia exists in several forms, and the most common form is an acute self-limited condition. Acquired pure red cell aplasia is often chronic and is associated with underlying disorders such as thymomas and autoimmune diseases.
The acquired chronic form of pure red cell aplasia is associated with thymomas and autoimmune disorders. Damage to erythroid progenitors or precursor cells appears to be immune and T-cell mediated. It is tempting to sue IVIG to regulate immune function so as to affect immunologic function. There is sufficient evidence that this is effective and a recent guideline recommends use of IVIG for red cell aplasia. Specific recommendations for routine use of IVIG were made for 7 conditions including acquired red cell aplasia (others were: acquired hypogammaglobulinemia (secondary to malignancy); fetal-neonatal alloimmune thrombocytopenia; hemolytic disease of the newborn; HIV-associated thrombocytopenia; idiopathic thrombocytopenic purpura; and posttransfusion purpura.)
Most studies that show response use “high-dose”. Total dose is administered IV but is graduated with low doses initially to monitor for anaphylaxis and other complications. Therefore, doses mentioned in package insert should be followed. Lower dosages/d but extended over 4 d are indicated in patients with fluid overload. Adult Dose Not to exceed 2 g/kg IV over 4 d
Zimmer J, Regele D, de la Salle H. Pure red-cell aplasia. N Engl J Med. Jun 24 1999;340(25):2004-5.
D . Anderson , K . Ali , V . Blanchette , M . Brouwers , S . Couban , P . Radmoor , L . Huebsch , H . Hume , A . McLeod , R . Meyer Guidelines on the Use of Intravenous Immune Globulin for Hematologic Conditions . Transfusion Medicine Reviews , Volume 21 , Pages S9 – S56 2007