Subject(s)
Hematoma/therapy , Hemophilia A/complications , Hemothorax/complications , Hemothorax/therapy , von Willebrand Diseases/complications , Adolescent , Anti-Bacterial Agents/therapeutic use , Blood Coagulation Factors/therapeutic use , Child, Preschool , Factor VIII , Hematoma/diagnostic imaging , Hemothorax/diagnosis , Humans , Male , Physical Therapy Specialty , Pneumonia/diagnosis , Pneumonia/drug therapy , Treatment Outcome , UltrasonographyABSTRACT
We describe the case of a previously healthy 8-year-old non-haemophilic boy who developed a factor VIII inhibitor of unknown origin. The symptoms of this disease were haemorrhages in the muscles of the right thigh, numerous bruises and a large haematoma of the right crus with subsequent tissue necrosis. Activated and non-activated prothrombin complex concentrates were administered in the therapy of the haemorrhages. To eliminate factor VIII inhibitor, the patient was treated first with prednisone, then immunoglobulin G and finally with a combination of prednisone and cycylophosphamide, without any effect. A total spontaneous remission was observed after 15 months from the beginning of the disease.
Subject(s)
Blood Coagulation Factor Inhibitors/blood , Factor VIII/antagonists & inhibitors , Hemophilia A/blood , Hemorrhage/etiology , Child , Hemophilia A/complications , Hemophilia A/drug therapy , Humans , Immunosuppressive Agents/therapeutic use , Male , Muscular Diseases/etiologyABSTRACT
INTRODUCTION: Immunoglobulin anti-D administration is one of several methods used in treating children with chronic immune thrombocytopenic purpura. Fc receptor blockade of the reticuloendothelial cell system and of mononuclear phagocytes is an important mechanism of the action of anti-D in ITP. Recently other possible mechanisms by which anti-D works in ITP have been considered. METHODS: The aim of this study was to obtain a better understanding of the effect of anti-D administration on cytokine, soluble cytokine receptors and platelet count in children with chronic ITP and to determine the pre-treatment plasma cytokine profile in this group of patients. Eighteen children with chronic ITP were examined. In our study the impact of anti-D on the cytokine network was evaluated by analysing serum levels of IL-6, IL-8, tumor necrosis alpha and soluble TNF receptors I and II by the EASIA method before and 1, 3, 20 and 40 h then seven days and one month after anti-D infusion. RESULTS: Anti-D caused a significant increase in platelet count 20 h postinfusion in 10 out of 18 children, 96 h postinfusion in three children and 168 h postinfusion in one child. The mean duration of the response was four weeks. A significant and rapid increase in plasma levels of IL-6, IL-8 and TNF-alpha was seen within 1 to 20 h after anti-D infusion. This increase was accompanied by a prolonged elevation of soluble TNF receptors. There was a significant correlation between TNF-alpha and IL-8, IL-8 and IL-6, TNF-alpha and sTNFRI, and sTNF receptors I and II. CONCLUSION: These data demonstrate that anti-D infusion caused changes in the cytokine network and raises the question of whether the therapeutic effectiveness of anti-D is related to its immunomodulating properties.
