Idiopathic thrombocytopenic purpura: beyond consensus

Idiopathic thrombocytopenic purpura (ITP) is the most common acquired bleeding disorder encountered by pediatricians. Most children with ITP have minimal bleeding and complete platelet count recovery within weeks to months. Therapy for ITP has ranged from close observation without medical intervention to aggressive management with corticosteroids, intravenous immunoglobulin G, or anti-D immune globulin. The topic of ITP has incited great debate among practitioners, and this debate prompted the development of ITP practice guidelines by the British Paediatric Haematology Group in 1992 and by the American Society of Hematology in 1996. A better understanding of the clinical course of, risk for significant bleeding in, and optimal evaluation and therapy of childhood ITP will require carefully designed, multicenter, clinical trials.     

Idiopathic thrombocytopenic purpura of childhood

Idiopathic thrombocytopenic purpura (ITP) is a benign hemorrhagic disorder characterised by peripheral thrombocytopenia and increased megakaryocytes in the bone marrow. The exact pathogenesis of ITP is not well understood. The adherence of viral induced immune complexes to the platelet membrane is thought to trigger the phagocytosis of damaged platelets by macrophages in the reticuloendothelial system. The role of platelet associated IgG in the pathogenesis of ITP is under investigation. Although spontaneous recovery is observed in 80–90% of patients, a short course of steroid therapy is recommended to reduce the duration of thrombocytopenia. The steroids however, have no influence on the course or outcome of the disease, and their possible role in reducing the incidence of intracranial hemorrhage (ICH) is unknown. Emergency management of patients presenting with signs and symptoms suggestive of ICH is essential to prevent the fatal outcome. Approximately 10–20% of patients develop chronic ITP. Splenectomy, considered the treatment of choice in these patients, is not always curative. The post-splenectomy sepsis also imposes a great risk for these individuals. Recent experience with intravenous immunoglobulin (IV IgG) treatment indicates that the splenectomy could safely be deferred, or even avoided in chronic ITP. The use of IV IgG in acute ITP is being investigated.     

Idiopathic thrombocytopenic purpura: long-term outcome]

BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) has usually an excellent prognosis even when no specific therapy is given. We studied, in 1996, the outcome of 87 patients with ITP observed between 1973 and 1995. PATIENTS AND METHODS: These 87 cases represented all the thrombocytopenic purpura observed during 23 years in the same pediatric unit not specialized in hematology. The outcome of these children was investigated in 1996 by phone or writing to the patients themselves or to their physician. Adequate information was obtained for 63 patients (72.4%). RESULTS: Two cerebral hemorrhages were observed, one was lethal while the other, observed after several years of course, healed completely. All these patients became free from clinical signs but 11 were not cured in 1996. None of these 63 patients had developed autoimmune disease. One patient died in a traffic accident, his thrombocytopenic purpura had been cured. CONCLUSION: In spite of two cerebral hemorrhages, ITP remains in the great majority of cases a benign disease not requiring grave therapeutic decisions.     

Idiopathic thrombocytopenic purpura (ITP): a historical odyssey

Purpura has been recognized since ancient times and its clinical syndromes were refined by important observations in the sixteenth, seventeenth and eighteenth centuries. It required the development of adequate microscopes in the nineteenth century, however, to recognize the platelet, leading to the recognition of the thrombocytopenic component of ITP. The twentieth century brought recognition of the pathophysiology of the disorder and further defined the clinical states and treatments for ITP. The latter half of the twentieth century has focussed on the autoimmune components of ITP, initially on the humoral immune aspects and more recently on dysregulation of cellular immunity.     

Idiopathic thrombocytopenic purpura: predictors of chronic disease.

We studied the extent to which patient characteristics influenced outcome in childhood idiopathic thrombocytopenic purpura in a historical cohort of 289 children over a 20 year period (1968-87). Outcome was classified as acute or chronic depending on whether the platelet count had returned to normal (150 X 10(9)/l) by six months after diagnosis. Fifty three cases (18%) had chronic idiopathic thrombocytopenic purpura. The likelihood of chronic disease was determined by logistic regression analysis of five patient variables: age, sex, season of onset of symptoms, history of recent viral illness, and duration of symptoms at presentation. A history of symptoms of greater than 14 days at presentation, adjusted for the other variables, was strongly predictive of chronic idiopathic thrombocytopenic purpura; the other variables did not significantly affect outcome. At 28 days after diagnosis 138 (47%) of the study cohort had normal platelet counts. Children whose platelet counts were less than 150 X 10(9)/l had a threefold risk of progressing to chronic idiopathic thrombocytopenic purpura, which increased to fivefold if counts were less than 50 X 10(9)/l. Two thirds of patients in the chronic group, irrespective of treatment, remained thrombocytopenic two years after diagnosis. We conclude that a history of symptoms for greater than two weeks at presentation is strongly predictive of chronic idiopathic thrombocytopenic purpura. If platelet counts are subnormal 28 days after diagnosis the risk of chronic idiopathic thrombocytopenic purpura is increased with prolonged thrombocytopenia being very likely if platelet counts remain low three months after diagnosis.     

Idiopathic thrombocytopenic purpura diagnosed during the second decade of life

OBJECTIVE: To retrospectively review our institutional experience of adolescents with idiopathic thrombocytopenic purpura (ITP). STUDY DESIGN: Medical record review of all patients diagnosed with ITP between the ages of 10 and 18 years seen at our center from January 1976 to March 2000. RESULTS: Data were collected from 126 patients. Of the evaluable 110 cases, 63 (57%) satisfied the criteria for chronic ITP, 30 (27%) for acute ITP, and 17 (15%) were uncertain. Sex distribution and mean ages were similar in all 3 groups. Platelet count at presentation was higher in patients with chronic ITP. Splenectomy was performed in 24 patients, with 17 (77%) of 22 having normal platelet counts at last follow-up. Outcome for the nonsplenectomized patients with chronic ITP included normalization of platelet count (n = 4), minimal or no bleeding without treatment (n = 29), treatment for ongoing symptoms (n = 5), and unknown (n = 1). Two patients died, 1 from intracranial hemorrhage and 1 from Escherichia coli sepsis and pulmonary hemorrhage. CONCLUSIONS: Patients 10 to 18 years of age with ITP are more likely than younger children to have chronic disease. Many patients with ITP recover without drug therapy or need for splenectomy. ITP in adolescents shares features of both childhood and adult ITP.     

Idiopathic thrombocytopenic purpura in children: should corticosteroids be given?

Life-threatening hemorrhages occur in about 2% of children with idiopathic thrombocytopenic purpura (ITP), the majority during the first month of illness. The administration of corticosteroids to patients with ITP has been shown to improve platelet economy mainly through reduction of phagocytosis in the spleen. Since the risk of a short course of steroids in low dosage is small, it seems reasonable to administer them to patients with ITP during the period of greatest risk.     

Idiopathic thrombocytopenic purpura in children. Apropos of 98 cases

The authors report a retrospective study of 98 cases of idiopathic thrombocytopenic purpura in children. The sex ratio was 1/1 and the mean age 5 years. A history of viral infection preceded the purpura in 45% of the cases. Sudden onset was observed in 81%. The platelet count was less than 20,000 mm3 in 78.5%. A follow-up was obtained in 79 patients: 76% had an acute disease. A spontaneous remission occurred in 88.6% of the cases, most of them in the first six months (49 cases/68), and in 10 cases/68 between 7 and 11 months after onset. Six patients presented one or two relapses prior to recovery. The illness became chronic in 11% of the patients despite prednisone therapy. Four patients were treated by splenectomy, in 3 cases with success. Two children died, one girl from cerebral haemorrhage and one boy from septicemia.