Hyperosmolar hyperglycemic nonketotic coma in Waldenström's macroglobulinemia associated with type II diabetes and complicated by pulmonary tuberculosis.

In this paper we describe a successfully treated case of hyperosmolar hyperglycemic non ketotic coma (HHNC). The HHNC was observed in a patient affected by Waldenström's macroglobulinemia, associated with type II diabetes and complicated by pulmonary tuberculosis. Hyperosmolar hyperglycemic nonketotic coma is a clinical condition with a high mortality rate associated with a severe increase in blood viscosity. This increase in blood viscosity justifies several clinical manifestations of the HHNC. We believe that an increase in the blood viscosity produced by the simultaneous presence of the single diseases mentioned above may have encouraged the development of hyperosmolar coma in the case reported

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 alteration

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

The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases

Evans syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune neutropenia. To better describe the characteristics and outcome of ES in adults, a survey was initiated in 2005. The data from 68 patients (60% of them women) fulfilling strict inclusion criteria for ES are reported. The mean age at time of ITP and/or AIHA onset was 52 plus or minus 33 years, both cytopenias occurred simultaneously in 37 cases (54.5%). ES was considered as "primary" in 34 patients (50%) but was associated with an underlying disorder in half of the cases, including mainly systemic lupus, lymphoproliferative disorders, and common variable immunodeficiency. All patients were given corticosteroids, but 50 of them (73%) required at least one "second-line" treatment, including splenectomy(n = 19) and rituximab (n = 11). At time of analysis, after a mean follow-up of 4.8 years, only 22 patients (32%) were in remission off treatment; 16 (24%) had died. In elderly patients, the risk of cardiovascular manifestations related to AIHA seems to be higher than the ITP-related risk of severe bleeding. In conclusion, ES is a potentially life-threatening condition that may be associated with other underlying autoimmune or lymphoproliferative disorders

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 \u2018terms and definitions of immune thrombocytopenia\u2019 (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 \u2018consensus\u2019 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 \u2018lines of therapy\u2019; 2. acceptance of the expression \u2018sequences of disease\u2019 for the indications of the respective four lines of treatment; 3I. practicability of splenectomy; 3Ib. acceptance, with only some exceptions, of a \u2018timing for elective splenectomy of 12 months\u2019; and 4a-d. \u2018goals of the four lines of therapy.\u2019 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

P17 - Early Menopause Influences Osteopenic or Osteoporotic Status in Postmenopausal Women: Preliminary Results from the Prof Project

AIMS: Italy has a considerable yearly incidence of osteoporotic fractures: about 300,000. In this context, preventive strategies are based mainly on early identification of people at higher risk of fractures and of clinical risk factors. Within the PROF (Prevention of Osteoporotic Fractures) project, i.e. a synergic effort of researchers and clinicians aimed at preventing osteoporotic fractures in Southern Apulia (Salento), a region with an increasing number of elderly people, we investigated the correlation between reduced physical activity and osteopenic or osteoporotic status in postmenopausal women. METHODS: During the years 2009–2010, 5665 postmenopausal women (mean age 62 years, range 39 to 86) underwent quantitative bone ultrasound (QUS) measurement at the heel and phalanx. Demographic and anamnestic data were recorded for all the patients, including BMI, nutrition, menopause, physical activity, previous fractures, familial fragility fractures. Three demineralisation categories were identified a priori: a) Demineralisation, when any T-score <−1.0 SD was observed; b) Severe demineralisation, whenever a T-score <−2.0 was observed, corresponding to a higher risk of fracture; c) Osteoporosis, whenever a T-score <−2.5±0.2 (for the heel) or T-score <−3.2±0.2 (for the phalanx) was observed. Descriptive statistical analyses were performed in order to assess the correlation between low physical activity (patients declaring themselves to be completely sedentary) and osteopenic or osteoporotic status. RESULTS: Of the 5665 women, demineralisation was observed in 4487 patients (79%), corresponding to severe osteopenia or osteoporotic status in 2823 women (50% of all the examined subjects) and frank osteoporosis in 846 patients (15%). In total, of the 1255 women with a clinical history of reduced physical activity, 1058 (84.3%) presented demineralisation corresponding at least to an osteopenic status. In addition, demineralisation typical of severe osteopenia or osteoporosis was diagnosed in 752 “sedentary” patients (60%), 309 of whom (25%) were frankly osteoporotic. CONCLUSIONS: In the PROF dataset, a sedentary lifestyle was found to be associated with an increased occurrence of osteopenic or osteoporotic status in postmenopausal women

Sensitization to propolis in 1255 children undergoing patch testing

To investigate the frequency and the features of contact sensitization to propolis in children, we patch tested 1255 consecutive children suspected of allergic contact dermatitis with propolis 20% petrolatum over an 8-year period (1995-2002). Positive responses were observed in 5.9% of patch-tested subjects, with a significantly higher frequency in boys than in girls. The dermatitis was localized mainly on the face, hands and limbs. A significant linear increase in the annual frequency of propolis allergy was noted. Confirming literature data, a significant association with balsam of Peru sensitivity was present in children reacting to propolis, linked to the presence of common constituents in the 2 substances. In conclusion, based on our data, we suggest that propolis should not be used in topical products for children because of its high sensitization rate at the paediatric age