Successful anti-D immunoglobulin therapy in refractory chronic idiopathic thrombocytopenic purpura showing reduction in thrombocytes even after splenectomy.
An 8 year old female child was treated with steroid hormones, anabolic steroid hormones and high dose gamma-globulin therapy for 2 years since being diagnosed with idiopathic thrombocytopenic purpura at the age of 6 years. Treatment produced only transient efficacy, and thrombocytopenia persisted. A scintigram taken 2 years after the onset of the disease using [125I]-labelled heated auto red cells revealed accumulation of radioactivities in the spleen, and therefore splenectomy was performed. However, thrombocytopenia (10 x 10(9)/L) developed again 3 weeks after the operation, for which she was treated again with high dose gamma-globulin with only transient recovery. Then, anti-D immunoglobulin was injected intramuscularly at 250 micrograms per dose for a total of four doses and the platelet count was restored to the normal range. Since then her platelet count has been maintained higher than 200 x 10(9)/L for these 11 months. The scintigram taken after splenectomy showed an accumulation of radioactivities in the liver. After administration of anti-D immunoglobulin, transient subclinical hemolysis appeared. The mechanism whereby anti-D immunoglobulin exerts the efficacy described here may be considered to be by blockage of Fc receptors of macrophages, as is the case for high dose gamma-globulin therapy. However, since the recovery in the platelet count persisted, it appears that changes in the immune system other than the above described mechanism have contributed to the recovery.
Shibuya
A
A
Department of Pediatrics, Saitama Medical School, Japan.
Danya
N
N
Shinozawa
T
T
D002648:Child; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007273:Injections, Intramuscular; D010976:Platelet Count; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood / Q000628:therapy*; D018029:Rho(D) Immune Globulin / Q000008:administration & dosage*; D013156:Splenectomy
D018029:Rho(D) Immune Globulin