Search

The clinical outcome, response to treatment, and occurrence of acute complications were retrospectively investigated in 308 primary autoimmune hemolytic anemia (AIHA) cases and correlated with serological characteristics and severity of anemia at onset. Patients had been followed up for a median of 33 months (range 12-372); 60% were warm AIHA, 27% cold hemagglutinin disease, 8% mixed, and 5% atypical (mostly direct antiglobulin test negative). The latter 2 categories more frequently showed a severe onset (hemoglobin [Hb] levels ≤6 g/dL) along with reticulocytopenia. The majority of warm AIHA patients received first-line steroid therapy only, whereas patients with mixed and atypical forms were more frequently treated with 2 or more therapy lines, including splenectomy, immunosuppressants, and rituximab. The cumulative incidence of relapse was increased in more severe cases (hazard ratio 3.08; 95% confidence interval, 1.44-6.57 for Hb ≤6 g/dL; P< .001). Thrombotic events were associated with Hb levels ≤6 g/dL at onset, intravascular hemolysis, and previous splenectomy. Predictors of a fatal outcome were severe infections, particularly in splenectomized cases, acute renal failure, Evans syndrome, and multitreatment (4 or more lines). The identification of severe and potentially fatal AIHA in a largely heterogeneous disease requires particular experienced attention by clinicians.

The clinical outcome, response to treatment, and occurrence of acute complications were retrospectively investigated in 308 primary autoimmune hemolytic anemia (AIHA) cases and correlated with serological characteristics and severity of anemia at onset. Patients had been followed up for a median of 33 months (range 12-372); 60% were warm AIHA, 27% cold hemagglutinin disease, 8% mixed, and 5% atypical (mostly direct antiglobulin test negative). The latter 2 categories more frequently showed a severe onset (hemoglobin [Hb] levels ≤6 g/dL) along with reticulocytopenia. The majority of warm AIHA patients received first-line steroid therapy only, whereas patients with mixed and atypical forms were more frequently treated with 2 or more therapy lines, including splenectomy, immunosuppressants, and rituximab. The cumulative incidence of relapse was increased in more severe cases (hazard ratio 3.08; 95% confidence interval, 1.44-6.57 for Hb ≤6 g/dL; P < .001). Thrombotic events were associated with Hb levels ≤6 g/dL at onset, intravascular hemolysis, and previous splenectomy. Predictors of a fatal outcome were severe infections, particularly in splenectomized cases, acute renal failure, Evans syndrome, and multitreatment (4 or more lines). The identification of severe and potentially fatal AIHA in a largely heterogeneous disease requires particular experienced attention by clinicians.

The possibility that daily sessions of music-guided slow breathing may reduce 24-h ambulatory blood pressure (ABP), and predictors of efficacy were explored in a randomized, placebo-controlled trial with parallel design.

Background.To investigate whether maternal anthropometric factors interact with one another or with other risk factors, thus modifying the risk of spontaneous preterm delivery.Methods.We carried out a case-control study of 230 spontaneous preterm births with intact membranes between 24 and 35 weeks gestation and 460 control term births. All the patients had prenatal care at the same institution. Logistic regression analysis was used to test for possible interactions adjusting for potential confounders.Results.A pre-pregnancy body mass index =19.5 Kg/m2(odds ratio (OR)=1.68, 95% confidence interval (CI)=1.20–2.38) and a rate of weight gain =0.37 Kg/week during the second and third trimesters (OR=2.4, 95% CI=1.69–3.42) were associated with an increased risk of spontaneous preterm delivery. The risk of spontaneous preterm delivery associated with a low second/third trimester weight gain was greater among patients with a body mass index =19.5 (OR=5.63, 95% CI=2.35–13.8) compared to those with a body mass index >19.5 (OR=2.45, 95% CI=1.60–3.75, adjusted pvalue for interaction=0.05). The risk of spontaneous premature delivery associated with a maternal pre-pregnancy weight =48 Kg was higher among smokers (OR=5.81, 95% CI=1.60–22.9) than among non-smokers (OR=2.4, 95% CI= 1.53–3.74, adjusted pvalue for interaction=0.05).Conclusions.The risk of spontaneous preterm delivery associated with a low pre-pregnancy body mass index is greater among patients with low rate of gestational weight gain during the second and third trimesters compared to those with a higher rate. The results of this study support the recommendation for increased rates of weight gain to patients with low body mass index compared to those with a higher body mass index.

To evaluate risk factors related to sociodemographic and clinical variables, oral contraception and sexual behavior of women with recurrent vulvovaginal candidiasis, we conducted a case-control study comparing 153 patients with recurrent vulvovaginal candidiasis with both asymptomatic women with negative vaginal cultures and patients with nonrecurrent symptomatic vulvovaginal candidiasis. In logistic regression analysis, patients with recurrent Candida vaginitis were more likely than negative controls to have used any contraceptive method in the year before evaluation, to have used antibiotics in the month preceding the visit, and to have a higher number of lifetime sex partners. Compared to patients with nonrecurrent Candida vaginitis, patients with recurrent infection were more likely to use oral contraception and to have a higher frequency of monthly intercourse. The proportion of recur rent disease attributable to the pill averages 11–12%. We conclude that oral contraceptives may influence the recurrence of symptomatic vulvovaginal candidiasis.

To estimate the risk for cerebral palsy in preterm infants in relation to the presence of meconium in the amniotic fluid (AF).

Introduction.During last years, a reduction in invasive aspergillosis (IA) incidence and mortality was observed in acute myeloid leukemia (AML) patients undergoing to induction phase of treatment. Instead, the incidence of IA and the attributable mortality rate (AMR) during the consolidation has not been recently evaluated. It is also uncertain whether posaconazole prophylaxis during induction chemotherapy affects IA on consolidation phase.

No relevant conflicts of interest to declare.

Background.During the last years, hematopoietic stem cell transplant from haploidentical donor is increasing by using the unmanipulated graft. We report the results of allogeneic bone marrow transplantation (BMT) from haploidentical donor primed with G-CSF in patients (pts) with high-risk acute myeloid leukemia (AML), who received an uniform TBF (Thiotepa, Busulfan, Fludarabine) conditioning regimen including antitymocyte globulin and an identical GVHD prophylaxis combining MTX+CSA, MMF and an anti-CD25 monoclonal antibody (Basiliximab).

No relevant conflicts of interest to declare.

Background.Similar probabilities of survival have been reported for patients transplanted from Matched Unrelated Donor (MUD), Umbilical Cord Blood (UCB) or Haploidentical (Haplo) donors as alternative hematopoietic stem cell sources. However, few studies have compared these results with those obtained in patients transplanted from HLA Id-siblings (Id-sib). Moreover, all reported studies are retrospective and the criteria of donor selection were not predefined. We report the intention to treat (ITT) analysis results on 238 patients with high-risk acute myeloid leukemia (AML) prospectively transplanted according to the policy of the Rome Transplant Network (RTN), a metropolitan transplant program established in Rome in 2006.

No relevant conflicts of interest to declare.

No relevant conflicts of interest to declare.

No relevant conflicts of interest to declare.

Patients and Methods.At the Rome Transplant Network, a JACIE accredited metropolitan transplant program, a matched-pair analysis has been conducted on 116 of 255 patients transplanted between January 2008 and December 2012. The patients transplanted from Id Sib (n=58) or Haplo related donors (n=58) were completely matched for the following features: patient age, gender, diagnosis (7 subgroups), disease phase (early: CR1+CR2; advanced: >CR2+active disease), myeloablative or reduced intensity conditioning regimen consisting of Thiotepa, i.v. Busulphan and Fludarabine (TBF) association, donor age and donor/recipient sex, AB0 and CMV combinations. As GVHD prophylaxis, all patients received the standard CSA and MTX combination with the addition of ATG, MMF and Basiliximab in Haplo bone marrow recipients. The transfusion policy, supportive care and antinfectious prophylaxis were identical for all patients.

OBJECTIVES

Autoimmune hemolytic anemia (AIHA) is a greatly heterogeneous condition both in terms of clinical presentation and response to treatment, usually classified as warm (WAIHA), cold (CAD), mixed, and atypical forms. The aim of this study was to identify predictors of outcome and response to therapy considering in particular the serological characteristics and the severity of anemia at onset. We evaluatedretrospectively 307 patients (112 M and 195 F, median age at diagnosis 63, range 1-97), diagnosed between 1978 and 2013 and followed-up for a median of 33 months (range 12-372); 60% of cases were WAIHA, 27% CAD, 8% mixed, and 5% atypical (14 DAT- and 1 DAT+ for IgA only). Hemoglobin values were lower in mixed (median 5.8, range 2-10.7 g/dL) atypical (6.2, 3-9), and in IgG+C3 DAT+ WAIHA (6.9, 2.9-11.5). Twenty-one subjects were diagnosed with Evans’ syndrome, the majority of them WAIHA, with a severe onset. Considering anemia at onset, 27% of cases had Hb levels <6, 36% Hb 6.1-8, 24% Hb 8.1-10, and 13% Hb>10 g/dL; the most severe cases were mainly mixed and atypical forms (P=0.0001).

A case of X-linked ichthyosis diagnosed antenatally by molecular analysis of fetal DNA is described. The diagnosis was made at 16 weeks gestation, following the finding of a maternal serum unconjugated estriol level lower than 0.1 MoM when performing a triple test. Fetal DNA was obtained from cultured amniocytes; two specific regions were amplified by polymerase chain reaction at 5′ and 3′ ends of the steroid sulfatase (STS) gene on Xp22.3 region. Analysis showed complete deletion of the STS gene on the distal tip of the X-chromosome short arm.

Invasive fungal infections (IFI) represent an important cause of mortality and morbidity in patients with Acute Myeloid Leukemia (AML) undergoing intensive chemotherapy. A prophylactic antifungal therapy is often administered during intensive chemotherapy, however the optimal antifungal prophylaxis protocol is still unknown. Amphotericin B lipid complex (Abelcet®) has been commonly used as a standard treatment for IFIs caused by Aspergillus and Candida, but its effectiveness in prophilaxis has not been clearly estabilished.From September 2010 to April 2011 we treated six patients with newly diagnosed AML using low dose amphotericin B lipid complex as antifungal prophilaxis. Patients observed were three females and 3 males, median age was 54 years (range 21–74 years) and they were all fit to receive intensive chemotherapy. Three patients older than 60 years received Fludarabine based chemotherapy regimen during both induction and consolidation. Three patients aged less than 60 years old were treated using a chemotherapy protocol based on Citarabine, Daunorubicin and Ethoposide association. One patient in this group also underwent BuCy conditioned autologous stem cell transplant. Amphotericin B lipid complex was administered intravenously at 100 mg once a day. Antifungal prophilaxis was started when the absolute neutrophil count was ≤ 500 cells/μ l and was continued until neutrophils recovery was ≥ 500 cells/μ l, without any evidence of IFI.Five patients did not experience any proven fungal infection during all treatment. Anyway one patient died during induction due to a severe bacterial lung infection. One patients discontinued antifungal prophylaxis due to extensive skin rash during the second infusion of the drug. Amphotericin B lipid complex was otherwise well tolerated by patients. One patient was diagnosed with lung aspergillosis infection by evidence of galattomannan positivity on BAL and a lung CT scan showing a single nodular escavated lesion on left upper lobe; subsequentely he was successfully treated with voriconazole.In our experience, Amphotericin B lipid complex showed to be an effective and safe antifungal prophylaxis for newly diagnosed AML patients. Further clinical studies are certainly required to obtain definitive data.No relevant conflicts of interest to declare.

Inflammatory myopathies are primary diseases of the striated muscles caused by an underlying autoimmune dysfunction. Dermatomyositis and polymyositis are the most common diseases of the striated muscle, skin and surrounding connective tissue. Usually the presentation includes proximal muscle weakness, inflammatory changes, creatine kinase elevation and skin rash. Although the cause of polymyositis/dermatomyositis is unknown, an autoimmune pathogenesis is strongly implicated and this provides the rationale for using immunosuppressant treatment. In the presteroid era prognosis was very poor, currently the mainstay treatment is based on high dose prednisone followed by different immunosuppressant and immunomodulating drugs as second and third line treatment for non responder patients. Autologous haemopoietic stem cell transplantation (HSCT) has been successfully employed in autoimmune diseases becoming a curative option for conditions with very poor prognosis, such as severe forms of systemic sclerosis, multiple sclerosis, and systemic lupus erythematosus. To our knowledge, 14 patients with polymyositis have been successfully treated with autologous bone marrow transplantation. The goal of PBSCT in polymyositis is to achieve remission of the systemic manifestations. Thus, we describe a case of dermatomyositis treated with high-dose chemotherapy and autologous peripheral blood stem cell transplantation (PBSCT), after a long history of conventional immunosuppressive therapy without advantage.In our centre, member of the Rome Transplant Network, we observed a 60-year old man with seronegative dermatomyositis, which began in 1996 with symmetric proximal muscle weakness. Dermatomyositis was diagnosed according to Bohan and Peter criteria with a 5/5 score. Despite the administration of five lines of different immunosuppressive treatments between 1996 and 2010, disease progressed with pharingo-laryngeal involvement causing dysphagia and aphonia and EMG evidence of severe myopathic changes. HSCs were successfully mobilized with cyclophosphamide 2 g and G-CSF 5 mg/kg/day. Time from diagnosis to transplantation was 15 years. Tiothepa 70 mg on day -5 and cyclophosphamide 3.5 g on day -3 and -2 were administered intravenously as conditioning regimen followed on day 0 by reinjection of the cells collected by leukapheresis. The number of CD34+ cells infused was 2.9 × 106 /Kg. All the procedures for PBSCT were well tolerated and the post-transplantation period was uneventful, except for an episode of neutropenic fever without bacteriological documentation that resolved under broad-spectrum antibiotics.After the PBSCT, we observed slow but progressive improvement in neurological symptoms and performance status. Currently, three months after transplantation, the patient is able to perform daily activities autonomously.Our case confirms the usefulness and safety of PBSCT in dermatomyositis. We are not able to tell which role high dosage of CSF have played in the clinical response. However our case reinforces the idea that PBSCT should be considered in the treatment of severe forms of dermatomyositis, even early during the disease course in order to reach the best response.No relevant conflicts of interest to declare.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) is a rare multi-system disease characterized by chronic progressive polyneuropathy and plasma-cell dyscrasia. A major diagnostic criterion for POEMS is elevation of circulating vascular endothelial growth factor (VEGF), which is believed to play a pathogenic role in this disease.We report 3 cases of POEMS syndrome treated with high-dose melphalan and autologous peripheral blood stem cell transplantation (PBSCT).The post-transplant course was uneventful other than the development of febrile neutropenia in 2 cases. Patients 1 and 2 presented a progressive improvement in strength, physical skills and peripheral neuropathy after few months. They are well with clinical stabilization after 4 and 6 years, respectively, from PBSCT. At 3 months from PBSCT, patient 3 presents progressive neurological improvement and reduction of organomegalies.Although treatment for POEMS is not standardized, disseminated disease require systemic therapy. No randomized controlled trials have been conducted, but patients with POEMS have been successfully treated with PBSCT since 1998. Our cases confirm its usefulness and safety and support the idea that, although delayed intervention maintains its efficacy, PBSCT could be considered early during the disease course to obtain the best response.No relevant conflicts of interest to declare.

Abstract 4507

Abstract 4504

Abstract 4303