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Primary immune thrombocytopenia (ITP), formerly known as immune thrombocytopenic purpura, is a disease in which clinical and therapeutic management has always been controversial. The ITP working group of the Spanish Society of Paediatric Haematology and Oncology has updated its guidelines for diagnosis and treatment of ITP in children based on current guidelines, literature review, clinical trials and member consensus. The primary objective was to lessen clinical variability in diagnostic and therapeutic procedures in order to obtain best clinical results with minimal adverse events and good quality of life., (Copyright © 2010 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.)

Deep vein thrombosis (DVT) has an estimated annual incidence of 0.07/10,000 children. Early diagnosis suspicion in the emergency department is important because it is a serious disease that, if untreated, can lead to a postthrombotic disease or a pulmonary thromboembolism. We report 2 cases of DVT whose diagnosis was made in the pediatric emergency department. Case 1 is a 9-year-old boy, evaluated with corticodependent nephrotic syndrome, who presented with pain in the lower left limb and increase in size of 48 hours' evolution suggestive of DVT. The elevation of D-dimer in the blood analysis and images from the Doppler ultrasound confirmed the diagnosis. His clinical evolution was good after beginning treatment with low molecular weight heparin. Case 2 is a 16-year-old adolescent, mother of a 1-year-old infant, who took oral contraceptives and was an occasional smoker, showed increased size and had pain in the lower left limb of a few hours' evolution. Deep vein thrombosis was suspected, and the diagnosis was confirmed by Doppler ultrasound. The evolution was favorable after beginning treatment with low molecular weight heparin. Although DVT is rare in children, early detection is important, requiring a detailed case history in the presence of edematous, painful, and hot limbs that are keys to the diagnostic suspicion. The imaging test and the laboratory tests will confirm the diagnosis, and anticoagulant treatment will prevent complications.

Pandemic influenza A (2009-H1N1) usually results in mild clinical illness, but in some individuals it can be life-threatening. There are no reports of this disease among paediatric patients with acute lymphoblastic leukaemia (ALL). We report ten consecutive patients with ALL and pandemic influenza treated in a single institution. Median age was 7 years (range: 3-12). All were treated with oseltamivir. There were no deaths. Two patients under intensive chemotherapy developed pneumonia and one required ventilatory support. ALL patients under maintenance treatment had mild disease. In conclusion, in our series only patients under intensive treatment developed a moderate to severe disease.

Idiopathic hypereosinophilic syndrome (HES) in children is a very rare disorder; certain clinical differences with adult HES have been described, with no pediatric case with the imatinib-responsive FIP1L1-PDGFRA fusion gene reported to date. The authors describe the clinical course of three children with HES in whom FIP1L1-PDGFRA fusion gene was studied and report the first child with this rearrangement.

Flow cytometry methods currently used for measuring neutrophil activation involve sample manipulation, which may result in cellular depletion and artifactual activation. To design a new methodology for measurement of neutrophil activation with minimal sample manipulation. Oxidative burst and CD 11b neutrophil expression were simultaneously assessed by a new no-lyse no-wash technique and a standard lyse-method in 10 pediatric patients with recurrent infections and two patients with chronic granulomatous disease (CGD). The new technique was based on nucleic acid staining to discriminate erythrocytes and debris without requiring physical separation. Both methods served equally to confirm or eliminate the diagnosis of CGD and leukocyte adhesion deficiency type 1. The values of baseline CD11b and oxidative burst obtained using the lysis method were significantly higher than those obtained by the no-lyse no-wash method. After activation, the lysis method resulted in higher neutrophil depletion (41%vs. 19%, P = 0.03). When compared with standard methods, neutrophil activation assessment by a no-lyse no-wash method resulted in lower neutrophil depletion and differences in oxidative burst and CD11b neutrophil values.

Purpura fulminans (PF) is an infrequent complication of varicella characterized by the progressive development of purpuric or painful ecchymotic lesions associated with biochemical alternations typical of consumption coagulopathy. Activation of coagulation is due to a marked and prolonged decrease in protein S, which is probably secondary to the formation of antiprotein S antibodies. The mechanism responsible for the synthesis of these autoantibodies is unknown. We present three cases of postvaricella PF and review the clinical and biochemical characteristics of this entity, as well as current diagnostic and therapeutic recommendations.

Introduction: Venous thrombosis is infrequent in paediatrics. A lot of prothrombotic risk factors have been described. Disturbances of coagulation are present in more than half of children with stroke. Leiden V factor mutation is emphasized as one of the most common genetic cause of deep venous thrombosis in Caucasian children and adults, which represents 20-25%, depending on series., Case Report: Two years-old girl with hypoxic-ischemic disease and West syndrome, which presents a deep venous thrombosis in both legs. This evolved to gangrene, requiring surgical exceresis of scar and amputation of 2nd, 3rd and 4th fingers of left foot. She also presented a right frontoparietal intraparenchima haemorrhagic stroke with tetraventricular bleeding and hydrocephalus. It required an external ventricular derivation and later a Ventricular-peritoneal valve., Conclusions: The coagulation study confirmed the Leiden V factor mutation in our patient. Later studies showed the same mutation in the father. The risk of recurrence and the severity of venous thrombosis indicate life antiaggregant treatment, currently maintained with aspirin.

Autoimmune hemolytic anemia (AIHI) is an infrequent disease in the pediatric age group. Its diagnosis is given by the direct antiglobulin test (DAT) or Coombs' test, which determines which type of globulin (IgG or complement) is the cause of the hemolysis. The type of globulin involved determines the etiology of AIHI, which is usually confirmed by positive results of other laboratory investigations such as cold agglutinin determination or the Donath-Landsteiner test. We present three cases of AIHI. DAT was positive to complement with diverse etiology: warm antibody with IgG-negative DAT, cold agglutinins associated with infectious mononucleosis, and Doth-Landsteiner antibodies. In all patients, empirical treatment with corticosteroids was initiated. The treatment was withdrawn or continued, depending on the final etiology of AIHI.

Case Report: Girl, aged 4, without antecedents who was admitted to our hospital for drowsiness and progressive sensorial depression. Within 24 hours the clinical picture deteriorated with partial seizures of the right hand side of the body and right hemiparesis. A brain CAT scan showed a left temporoparietal parenchymatous haematoma with collapse of the left lateral ventricle and moderate obliteration of the basal cisterns. MR angiography and cerebral arteriography displayed images that were compatible with thrombosis of the superior sagittal, the left transverse and the sigmoid sinuses. The patient was heterozygotic for the G A mutation in position 20210 of the prothrombin gene, which is linked with a high risk of thrombosis. She was given heparin intravenously, but continued to display endocranial hypertension and tissue ischemia with partial response to hyperosmolar agents and barbituric coma. She was therefore submitted to selective catheterization of the superior sagittal sinus and continuous local fibrinolysis with urokinase for 72 hours. The outcome was satisfactory, with repermeabilisation of the thrombosed sinuses and a good clinical response with no complications. At present the patient has functional paresis of the right hand and receives treatment with oral anticoagulants., Conclusions: We advocate the use of early local fibrinolytic treatment with urokinase in children affected by thrombosis of the venous sinuses who do not respond to treatment with sodium heparin. We consider it necessary to include the molecular study of the G20210A mutation of the prothrombin gene in screening for prothrombotic risk factors in small children

Objective: Thrombotic thrombocytopenic purpura (TTP) or Moschovitz' syndrome is rare and is even rarer in childhood. Clinically, it is characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. The etiology is still unknown, although different factors such as large von Willebrand factor multimers and prostacyclin have been implicated. The acute form is more frequent, and in most cases the course is fulminant if treatment is not initiated. Laboratory data typically reveal hemolytic anemia, with schistocytes on the peripheral smear, diminished serum haptoglobin, and thrombocytopenia., Material and Methods: We present the clinical cases of two children, aged 4 and 7 respectively, with TTP, but with different evolution and treatment. Evolution was favorable in both patients. The first child recovered spontaneously. In the second plasmapheresis was required and produced remission of all the symptomatology. Normality has been maintained for 36 and 24 months respectively, and the children have presented no clinico-biological alterations.