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Available data have proved insufficient to develop consensus recommendations on the prevention of thrombosis and bleeding in myelofibrosis (MF). We evaluated the incidence and risk factors of vascular complications in 1613 patients from the Spanish Myelofibrosis Registry. Over a total of 6981 patient-years at risk, 6.4% of the study population had at least one thrombotic event after MF diagnosis, amounting to an incidence rate of 1.65 per 100 patient-years. Prior history of thrombosis, the JAK2 mutation, and the intermediate-2/high-risk International Prognostic Scoring System (IPSS) categories conferred an increased thrombotic risk after adjustment for the risk-modifying effect of anti-thrombotic and cytoreductive treatments. History of thrombosis and the JAK2 mutation allowed us to pinpoint a group of patients at higher risk of early thrombosis. No decreased incidence of thrombosis was observed while patients were on anti-thrombotic or cytoreductive treatment. An increased risk of venous thrombosis was found during treatment with immunomodulatory agents. A total of 5.3% of patients had at least one episode of major bleeding, resulting in an incidence rate of 1.5 events per 100 patient-years. Patients in the intermediate-2/high-risk IPSS categories treated with anti-coagulants had an almost sevenfold increased risk of major bleeding. These findings should prove useful for guiding decision-making in clinical practice.

Atopic dermatitis is an allergic T-cell mediated skin inflammation. Staphylococcus aureus colonization is very common in cutaneous atopic dermatitis lesions. The cutaneous lymphocyte-associated antigen (CLA) is a T cell skin homing receptor that defines T lymphocytes associated with the cutaneous immune response.To study whether CLA+ T cells from atopic dermatitis children present a selective expression for Staphylococcus aureus-related TCR Vbeta segments.Peripheral blood T cells were stained with HECA-452 (anti-CLA) and a panel of TCR Vbeta specific monoclonal antibodies and analysed by flow cytometry.Atopic dermatitis patients have a higher percentage of circulating CLA+ CD3+ lymphocytes compared with healthy controls. Patients with active atopic dermatitis during the study expressed a higher percentage of cells positive for the TCR Vbeta2 and Vbeta5.1 segments in the CLA+ but not in the CLA- subset. These TCR Vbetas are recognized by staphylococcal superantigens. Moreover, there was an increased percentage of HLA-DR+ expression by CLA+ Vbeta5.1+ T cells in patients with active atopic dermatitis, but those patients whose eczema was inactive had very similar values to healthy controls regarding TCR Vbeta and HLA-DR phenotype in circulating CLA+ T lymphocytes.Our data indicate that circulating skin-homing T cells of patients with active atopic dermatitis contain an increased percentage of cells bearing TCR Vbeta segments related with Staphylococcus aureus. Staphylococcus superantigens may therefore trigger expansion or at least circulation of appropriate CLA+ T cells.

Cytarabine (Cyt) is an antimetabolite used primarily in the treatment of leukemia, and both immediate and delayed hypersensitivity reactions have been reported. We studied a 9-year-old girl with lymphoblastic leukemia, who developed three anaphylactoid reactions during Cyt treatment courses over a 1-year period. Three years later, Cyt was required again. Although a skin test was negative to Cyt at the concentration of 4 micrograms/ml, we decided on placebo-controlled administration of the drug. The Cyt was well tolerated, and urine values of N-methylhistamine showed no important variations throughout this period compared to those during the placebo administration. Skin tests carried out 14 days after the study were positive at the concentration of 4 micrograms/ml. The history of different episodes of allergic reactions to Cyt, the last one being the most severe, indicated the possible participation of an immediate hypersensitivity phenomenon, but because no studies had been carried out initially, we could not establish the presence of IgE antibodies. These results indicate that good tolerance existed after the control administration procedure. The long interval, 3 years, between the allergic episode and our protocol and the appearance of a positive skin test 14 days after the protocol indicated that the subject had lost sensitivity and become resensitized after the controlled administration procedure.

Idiopathic pulmonary hemosiderosis (IPH) is characterized by repeated episodes of intraalveolar bleeding, hemosiderin deposition in alveolar macrophages, and subsequent development of pulmonary fibrosis and severe anemia? This disease predominantly affects children; the age of occurrence ranges from 4 months to 62 years, although cases in newborn infants have been described. 2 The pathologic mechanism is not well understood, but some evidence supports an immunopathologic basis. The association of IPH with hypersensitivity to cow's milk proteins was first reported by Heiner and Sears 3 who described patients with recurrent pulmonary disease, changing roentgenographic manifestations, hemoptysis, iron-laden macrophages in gastric or bronchial washings, iron deficiency anemia, and multiple precipitins to cow's milk. Additional studies have shown peripheral blood eosinophilia; alveolar deposits of IgG, IgA. and C3; circulating immune complexes: and delayed hypersensitivity to cow's milk proteins?- 5 We studied a newborn infant with IPH and myocardial involvement in whom the administration of cow's milk proteins induced peripheral eosinophilia and the release of histamine and eosinophilic cationic protein (ECP) in peripheral blood and bronchoalveolar lavage (BAL) and the release of histamine in urine.

Urinary excretion of acid mucopolysaccharides was determined in seven children with Hurler's disease and in 1000 apparently normal children using a simple turbidity method. All the seven patients with Hurler's syndrome gave values of mucopolysaccharides which were considerably higher than those obtained in the control groups. In the screening test, 32 children with various diseases showed a marked increase in mucopolysaccharides excretion. It must be emphasized that one of those children —aged 1 month at the time of the analysis—was diagnosed later on as a new case of Hurler's syndrome.

In the past few years there has been a resurgence of interest in the protein composition of human milk. Up to now the influence of maternal diet and of the mothers' nutritional status on the protein composition of human milk have not been fully clarified. We have evaluated the relationship between the mothers' socioeconomic status and weight and the protein composition of human milk. Protein fractions were determined by a polyacrylamide gel electrophoresis method in 181 samples of human milk obtained from voluntary donors. Samples were classified according to the time of lactation and in relation to the socioeconomic status and to the weight of the lactating women. Total protein and non-protein nitrogen decreased with advancing lactation but there were no differences among the socioeconomic and weight groups of mothers who were considered. beta- and kappa-caseins fell during lactation and beta-casein was significantly increased in the milk of the upper socioeconomic class with respect to that of the low one. alpha-lactalbumin increased from transitional to mature milk (16-30 d) and then declined. The milk from the low socioeconomic group presented the lowest levels of this protein. Lysozyme increased during lactation, whereas lactoferrin decreased. Both proteins were significantly influenced by the mothers' socioeconomic status; the highest concentrations for these proteins were found in the milk of the low socioeconomic group. Deficit or excess of mothers' weight did not influence the levels of the different protein fractions of human milk.

A review of the diagnostic methods of pertussis (clinical, radiological, laboratoryy: 42 case reports in a period of 2 1/2 years, being all hospitalized children with pertussis, aged from 23 days to 7 years. A review of the treatment with hyperimmune globulin, antibiotics, symptomatic treatment, and the results obtained, is presented.

The changes in the absolute and relative contents of alpha- and kappa-caseins, lactoferrin, alpha-lactalbumin, serum albumin and lysozyme in human milk have been studied through the period of lactation. Protein fractions of 209 samples were analyzed by a discontinuous polyacrylamide gel electrophoresis method. beta- and kappa-caseins decreased from colostrum to mature milk although their relative percentages remained constant. They accounted for 12-15 and 9-13% of the total protein in human milk, respectively. Lactoferrin decreased in absolute and relative amounts with advancing lactation. This protein represented 32-19% of the human milk proteins. alpha-Lactalbumin slightly decreased from colostrum to transitional milk but there was an increase in mature milk by 16-30 days. The percentages of this protein in colostrum and mature milk were approximately 23 and 30%, respectively. Serum albumin also decreased with advancing lactation, but the differences between transitional and mature milk were not statistically significant. Lysozyme increased from colostrum to mature milk both in relative and absolute amounts. Colostrum contained about 262 micrograms/ml, and mature milk 1,246 micrograms/ml, representing 1.5 and 12.1% of total milk proteins.