Investigating factors associated with adherence behaviour in patients with chronic myeloid leukemia: an observational patient-centered outcome study.

BACKGROUND: Optimal adherence to imatinib therapy is of paramount importance to maximise treatment effectiveness in patients with chronic myeloid leukaemia (CML). The main objective of this study was to investigate patient-reported personal factors associated with adherence behaviour. METHODS: Analysis was conducted on 413 CML patients receiving long-term therapy with imatinib. Adherence behaviour was measured with the Morisky Medication Adherence Scale and personal factors investigated included: quality of life, perceived social support, fatigue, symptom burden, psychological wellbeing and desire for additional information. Key socio-demographic and treatment-related factors were also taken into account. Univariate and multivariate logistic regression analyses were used to investigate factors associated with optimal adherence to therapy. RESULTS: In all, 53% of patients reported an optimal adherence behaviour. The final multivariate model retained the following variables as independent predictors of optimal adherence to therapy: desire for more information (ref. no), odds ratio (OR)=0.43 (95% confidence interval (CI), 0.29-0.66; P<0.001), social support (higher score representing greater support), OR=1.29 (95% CI, 1.11-1.49; P<0.001) and concomitant drug burden (ref. no), OR=1.82 (95% CI, 1.18-2.80; P=0.006). CONCLUSION: This study suggests that a higher level of social support, satisfaction with information received and concomitant drug burden are the main factors associated with greater adherence to long-term imatinib therapy.

Effect of rituximab on clinical and laboratory features of antiphospholipid syndrome: a case report and a review of literature.

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by a hypercoagulable state related to persistently elevated levels of antiphospholipid antibodies (aPL). Current treatment for APS is only partially effective and new therapies are strongly needed. We report on a case of a 50 years old man with APS who suffered from recurrent thromboembolic episodes despite conventional anticoagulant treatment. Eight years after the first thrombotic manifestation he was diagnosed with a large B cell non-Hodgkin lymphoma. Treatment with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) plus rituximab was started with partial clinical remission of lymphoma and normalization of aPL levels with a three years follow-up period free of thrombotic episodes.A review of the literature revealed that only 12 case reports on the use of rituximab in patients with primary, secondary and catastrophic APS have been published. Current knowledge clearly suggests the need for clinical trials to evaluate the effect of rituximab in the treatment of resistant APS.

Serum interleukin-8 levels in thalassemia intermedia.

In this study we determined serum IL-8 levels in 18 untransfused patients with beta-thalassemia intermedia and in 14 subjects affected by HbH disease. As reported in polytransfused homozygous beta-thalassemia, untransfused beta-thalassemia and HbH disease show significantly (p < 0.005) higher serum IL-8 levels than normal controls. Our data suggests that there could be an intrinsic cause for the IL-8 increase in thalassemia intermedia. We think than the hyperactivity of thalassemic macrophages related to chronic hemolysis is the main cause for the increment in cytokines, such as IL-8, found in thalassemic syndromes.

Interferon-alpha 2a therapy in CML: disappearance of BCR/ABL transcript in a case of long-lasting continuous cytogenetic conversion.

Seventy months after diagnosis, minimal residual disease is undetectable in a patient with Philadelphia chromosome-positive chronic myelogenous leukemia (CML) in long-lasting continuous cytogenetic conversion (CCC), achieved through alpha 2a-interferon (IFN-alpha) therapy. Fluctuating molecular remission, evaluated with the two-stage reverse transcriptase-polymerase chain reaction (RT-PCR) with nested primers, has persisted for two years at the maximum tolerable dose of IFN alpha (1.5 x 10(6) IU per day).

Repeated PCR in CML during IFN-alpha therapy.

Repeated PCR analysis was performed on bone marrow and/or peripheral blood samples from 4 CML patients in complete cytogenetic remission during treatment with IFN-alpha. Two patients became PCR-negative. One was negative for the analyses carried out from the 9th to the 30th months, but reverted to PCR positivity 10 months after IFN was reduced from 1.5 x 10(6) IU/day to 1 x 10(6) IU and given on alternate days. Although the dose was again raised to 3 x 10(6) IU/day, 8 months later her peripheral blood cells were still PCR-positive, but remained persistently Ph'-negative. Another patient became PCR-negative at the 42nd month and remained so at the last analysis performed 3 months later. Two patients were persistently PCR-positive. Cytogenetic relapse was documented in both, in one while still on full therapy. Ph'-positive metaphases reappeared in the other patient 7 months after discontinuing IFN-alpha therapy.

Incidence and clinical study of ectopic erythropoiesis in adult patients with thalassemia intermedia.

We carried out total body computerized tomography (CT) studies and examined the retrospective clinical data of 29 adult patients with thalassemia intermedia (TI) to evaluate the incidence, features and pathogenesis of ectopic erythropoiesis (EE), located chiefly at the paravertebral gutters in the thorax, was present in 65.5% of the patients; 15% of them had severe clinical complications. We found a clear relationship between EE development and early presentation age of thalassemia, splenectomy and the presence of 100% fetal hemoglobin. The frequent occurrence of EE suggests that CT screening of patients with thalassemia intermedia should be mandatory. We also recommend radiotherapy as a preventive measure for the clinical complications of thalassemic patients with EE.

[Complete atrioventricular canal associated with a tetralogy of Fallot. Results of surgical treatment. Apropos of 4 cases]. / Canal atrio-ventriculaire complet associé à une tétralogie de Fallot. Résultats de la cure chirurgicale. A propos de quatre observations.

Complete atrioventricular canal with tetralogy of Fallot is a rare occurrence (8%). Diagnosis rests on the association with Down syndrome and on data from EKG, ultrasonography and angiography. The high operative risk explains why radical surgery is done at an older age (9 years) than in the usual form of complete atrioventricular canal without associated anomalies (33 months) and why antecedent surgical palliation with anastomotic procedures is so frequent (68% of the cases reported in the medical literature). The same surgical procedure was done in all patients: closure of the defects with two separate patches lined with pericardium, suture of the mitral cleft and relief of the infundibular and pulmonary obstruction without valvulation.

[Isolated communication between the left atrium and the coronary sinus (author's transl)]. / Comunicazione atrio sinistro seno coronarico isolata. Raro caso corretto con tecnica personale.

The communication between the coronary sinus and the left atrium is a very exceptional case. In this work the AA. report the difficulties of the pre and peroperating diagnosis, above all because the coronary sinus, in this case, is not enlarged, but usual. The identification of the malformation, that is also isolated, is difficult also at the surgery, if you don't make a left atriotomy. A personal operative method of correction is reported.