Practical therapy for primary autoimmune hemolytic anemia in adults.

BACKGROUND AND AIM: Autoimmune hemolytic anemias (AIHA) constitute a rare and heterogeneous group of diseases whose therapy differs according to the type of antibody involved in the genesis of the disease and the existence or not of an identified cause. With the aim of providing a practical guide for the therapy of AIHA, we summarize the emergency therapy and general measures habitually used in all forms of AIHA, as well as the specific treatment of the most frequent primary forms of AIHA: primary warm AIHA and AIHA from cold agglutinin disease (AIHA from CAD). We discuss the dependence of the treatment of the secondary forms on their underlying causes and the changes in the treatment of the primary forms in recent years. METHODS: We examined the options available for the treatment of primary warm AIHA and AIHA from CAD. RESULTS: We found many differences and only one similarity in their treatment. DISCUSSION: The differences, particularly due to the non-responsiveness of AIHA from CAD to many treatments useful for primary warm AIHA, such as steroids, splenectomy and immunosuppressive agents, must be considered in the face of each, single case of AIHA. Preliminary identification of the type of antibody involved in the genesis of the disease and careful exclusion of a secondary form are particularly important. Rituximab plays a central role in the treatment of primary warm AIHA and AIHA from CAD.

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.

Goals defining therapy for primary immune thrombocytopenia in adults.

: Discordances existing between the two most influential guidelines on the treatment of immune thrombocytopenia, the 2010 International Consensus Report and the 2011 America Society of Hematology guideline, continue to be reflected by the heterogeneity of clinical practice of the physicians who treat this disease. Aimed at overcoming these discordances, we hypothesized that they could be ascribed to nonshared treatment goals. We classify the indications for and goals of the various possible treatments available for adults with primary immune thrombocytopenia according to the line of treatment and the phase of disease. The resulting classification is useful for recognizing the appropriateness of a chosen treatment in individual patients. The classification proposed here could constitute the basis for greater agreement among future guidelines and, in the meanwhile, could help less expert physicians recognize the appropriateness of a treatment to choose in the single patient.

A general model of dioxin contamination in breast milk: results from a study on 94 women from the Caserta and Naples areas in Italy.

BACKGROUND: The Caserta and Naples areas in Campania Region experience heavy environmental contamination due to illegal waste disposal and burns, thus representing a valuable setting to develop a general model of human contamination with dioxins (PCDDs-PCDFs) and dioxin-like-PCBs (dl-PCBs). METHODS: 94 breastfeeding women (aged 19-32 years; mean age 27.9 ± 3.0) were recruited to determine concentrations of PCDDs-PCDFs and dl-PCBs in their milk. Individual milk samples were collected and analyzed according to standard international procedures. A generalized linear model was used to test potential predictors of pollutant concentration in breast milk: age, exposure to waste fires, cigarette smoking, diet, and residence in high/low risk area (defined at high/low environmental pressure by a specific 2007 WHO report). A Structural Equation Model (SEM) analysis was carried out by taking into account PCDDs-PCDFs and dl-PCBs as endogenous variables and age, waste fires, risk area and smoking as exogenous variables. RESULTS: All milk samples were contaminated by PCDDs-PCDFs (8.6 pg WHO-TEQ/98g fat ± 2.7; range 3.8-19) and dl-PCBs (8.0 pg WHO-TEQ/98g fat ± 3.7; range 2.5-24), with their concentrations being associated with age and exposure to waste fires (p < 0.01). Exposure to fires resulted in larger increases of dioxins concentrations in people living in low risk areas than those from high risk areas (p < 0.01). CONCLUSIONS: A diffuse human exposure to persistent organic pollutants was observed in the Caserta and Naples areas. Dioxins concentration in women living in areas classified at low environmental pressure in 2007 WHO report was significantly influenced by exposure to burns.

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.

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.

Leishmania infection in a 51-year-old woman with sarcoidosis: case report.

Patients with sarcoidosis are at risk of opportunistic infections both from the sarcoidosis itself and from steroid therapy. Steroid therapy by reducing cell-mediated immunity increases the risk of infection with intracellular organisms. We present a case of Leishmania infection in a 51-year-old Italian woman receiving prednisone for sarcoidosis. The patient lives in Liguria, a region in the northwest of Italy that is considered highly endemic for leishmaniasis. We would postulate that even though leishmaniasis is seen relatively infrequently in connection with sarcoidosis, the occurrence of this opportunistic infection is possible in patients living in areas endemic for such protozoa.