Report of a 'consensus' on the lines of therapy for primary immune thrombocytopenia in adults, promoted by the Italian Gruppo di Studio delle Piastrine.

Despite the publication in 2009 of a paper on 'terms and definitions of immune thrombocytopenia' (ITP), some unresolved issues remain and are reflected by the disagreement in the treatment suggested for primary ITP in adults. Considering that these disagreements could be ascribed to non-shared goals, we generated a 'consensus' on some terms, definitions, and assertions useful for classifying the different lines of treatment for primary ITP in adults according to their indications and goals. Agreement on the appropriateness of the single assertions was obtained by consensus for the following indicators: 1. classification of four 'lines of therapy'; 2. acceptance of the expression 'sequences of disease' for the indications of the respective four lines of treatment; 3I . practicability of splenectomy; 3Ib . acceptance, with only some exceptions, of a 'timing for elective splenectomy of 12 months'; and 4a-d . 'goals of the four lines of therapy.' On the basis of the consensus, a classification of four lines of treatment for primary ITP in adults was produced. In our opinion, this classification, whose validity is not influenced by the recently published new guidelines of the American Society of Hematology (ASH) and reviews, could reduce the disagreement that still exists regarding the treatment of the disease.

Reviewed diagnosis of primary and secondary immune thrombocytopenic purpura in 79 adult patients hospitalized in 2000-2002.

Despite the accepted distinction between primary and secondary immune thrombocytopenic purpura (ITP), a systematic analysis of the incidence of secondary ITP is not available. The present study was aimed at verifying the frequency and, consequently, the approximate rates of prevalence and incidence of secondary ITP and analysing its clinical and laboratory characteristics in patients needing ordinary hospital treatment for ITP. The study was based on 79 consecutive, adult ITP patients admitted to three Italian hospitals in 2000-2002. Using data collected in a previous study on the appropriateness of hospital management of ITP, we evaluated the frequency of secondary ITP, with the diagnosis formulated on the basis of new acquisitions, derived its rates of prevalence and incidence, and examined the available clinical and laboratory parameters. At our case review, a diagnosis of secondary ITP could be formulated in 38% of the 79 patients. This frequency was significantly higher than that determined at the time the patients were discharged from hospital (13.9%) (P = 0.000). The derived rates of prevalence and incidence of secondary ITP in the general population were, respectively, 2.3 and 1.23 per 100 000 inhabitants per year. In comparison with patients with primary ITP, those with a secondary form more frequently had spleen enlargement (P = 0.000), hepatomegaly (P = 0.001) and lower haemoglobin values (P = 0.005). The high frequency of secondary ITP must be mainly attributed to the currently available knowledge about the nature of some forms of ITP. Particular contributors to the high frequency were cases secondary to infections and those observed in patients who had undergone bone marrow or solid organ transplantation. Some clinical and laboratory alterations appear to be more frequent in secondary ITP than in primary ITP. However, the importance that the identification of particular forms of ITP, such as those secondary to Helicobacter pylori or hepatitis C virus infections, has on the choice of treatment suggests that these conditions must be ascertained independently of the presence or absence of clinical and laboratory alterations.

Appropriate hospital management of adult immune thrombocytopenic purpura patients in major Italian institutions in 2000-2002: a retrospective analysis.

The treatment of immune thrombocytopenic purpura (ITP) still offers challenges to clinicians and health professional organizations, despite recommendations provided by international guidelines. In order to improve the care of patients with ITP, it is useful to understand how often such patients receive appropriate treatment and if common errors occur that could be avoided. We retrospectively analyzed all the clinical records between 1 January, 2000 and 31 December, 2002 of patients receiving an ICD-9-CM diagnosis code of 287.3 in three hospitals in northern Italy. We examined whether management strategies of adult men and nonpregnant women with ITP were consistent or not with the guidelines provided by the American Society of Hematology. The ITP diagnosis was confirmed in 120 of 169 patients (71%). Reasons for admission were: medical treatment for ITP (51.7%), medical or surgical treatment of ITP-associated disorders (30.8%), elective splenectomy (15.8%) and diagnosis or observation of ITP (1.7%). Hospital admission resulted appropriate in 78.1% of cases. Therapeutic interventions were appropriate in 100% of cases for glucocorticoid treatment, 86.4% for splenectomy, 47.7% for high-dose immunoglobulins, 40.9% for prophylaxis against bleeding before splenectomy, 33.3% for high-dose glucocorticoid treatment, and 19% for prophylaxis against bacterial infections before splenectomy. Platelet transfusions as treatment for bleeding were appropriate in only 20% of cases. Confirming the usefulness of the American guidelines for ITP, our data suggest that there are important areas of inappropriate management of the disease, which could be corrected by adopting quality improvement programs and studies.

Wheat IgE-mediated food allergy in European patients: alpha-amylase inhibitors, lipid transfer proteins and low-molecular-weight glutenins. Allergenic molecules recognized by double-blind, placebo-controlled food challenge.

BACKGROUND: Three main problems hamper the identification of wheat food allergens: (1) lack of a standardized procedure for extracting all of the wheat protein fractions; (2) absence of double-blind, placebo-controlled food challenge studies that compare the allergenic profile of Osborne's three protein fractions in subjects with real wheat allergy, and (3) lack of data on the differences in IgE-binding capacity between raw and cooked wheat. METHODS: Sera of 16 wheat-challenge-positive patients and 6 patients with wheat anaphylaxis, recruited from Italy, Denmark and Switzerland, were used for sodium dodecyl sulfate-polyacrylamide gel electrophoresis/immunoblotting of the three Osborne's protein fractions (albumin/globulin, gliadins and glutenins) of raw and cooked wheat. Thermal sensitivity of wheat lipid transfer protein (LTP) was investigated by spectroscopic approaches. IgE cross-reactivity between wheat and grass pollen was studied by blot inhibition. RESULTS: The most important wheat allergens were the alpha-amylase/trypsin inhibitor subunits, which were present in all three protein fractions of raw and cooked wheat. Other important allergens were a 9-kDa LTP in the albumin/globulin fraction and several low-molecular-weight (LMW) glutenin subunits in the gluten fraction. All these allergens showed heat resistance and lack of cross-reactivity to grass pollen allergens. LTP was a major allergen only in Italian patients. CONCLUSIONS: The alpha-amylase inhibitor was confirmed to be the most important wheat allergen in food allergy and to play a role in wheat-dependent exercise-induced anaphylaxis, too. Other important allergens were LTP and the LMW glutenin subunits.

Lipid transfer protein and vicilin are important walnut allergens in patients not allergic to pollen.

BACKGROUND: Walnut is the most common cause of allergic reactions to tree nuts, as reported by large population studies. Two major allergens of walnut have been identified up until now: a 2S albumin and a vicilin-like protein. OBJECTIVE: This study was designed to identify the walnut major allergens in the Italian population and to compare the walnut IgE-binding profile in patients with or without pollen allergy. METHODS: We selected 46 patients either with oral allergy syndrome confirmed by open oral challenge or with systemic symptoms after ingestion of walnut. These patients' sera were used for the immunoblotting of walnut extract; the identified allergens were purified by HPLC and sequenced. A peach-walnut cross-inhibition study was then performed. RESULTS: The only major allergen recognized by our study population was a 9-kd lipid transfer protein (LTP), recognized by 37 patients. Two other minor allergens of approximately 9-kd molecular weight, both belonging to the vicilin family, were recognized by 10 patients. IgE binding to walnut LTP was completely inhibited by peach LTP. CONCLUSION: In Italian patients with walnut allergy confirmed by documented history of severe systemic reactions or by open oral food challenge, the major allergen is an LTP. The sensitization to this protein seems to be secondary to the sensitization to peach LTP, which acts as the primary sensitizer. LTP and vicilins were able to sensitize patients not allergic to pollen.

Clinical role of lipid transfer proteins in food allergy.

Lipid transfer proteins are widespread plant food allergens, highly resistant to food processing and to the gastrointestinal environment, which have recently been described as true food allergens in the Mediterranean area, where they have been associated with severe allergic reactions to foods in patients without pollen allergy. In this review we analyze their molecular structure, biological function, and clinical relevance in food allergy.