Ida-FLAG plus imatinib mesylate-induced molecular remission in a patient with chemoresistant Ph1+ acute myeloid leukemia

Imatinib mesylate is a potent, selective inhibitor of the tyrosine kinase activity of bcr-abl,which is now established as the state-of-the-art treatment for chronic, accelerated or even blastic phase of Philadelphia-positive [Ph(1)(+)] chronic myelogenous leukemia. It is also active in Ph(1)(+) acute lymphoblastic leukemia, but its role in Ph(1)(+) acute myeloid leukemia (AML) is less well investigated. We report here a patient with chemoresistant Ph(1)(+) AML, who responded promptly to one cycle of Ida-FLAG second-line chemotherapy by achieving complete morphologic, immunophenotypic, and cytogenetic remission but not a molecular one. The addition of imatinib mesylate led to a molecular remission, which is sustained for 10 months so far.     

Giant extra gastrointestinal stromal tumor of lesser omentum obscuring the diagnosis of a choloperitoneum

INTRODUCTIONHerein we present an extremely rare case of a giant extra gastrointestinal stromal tumor (EGIST) of the lesser omentum obscuring the diagnosis of a choloperitoneum.PRESENTATION OF CASEA 79 years old female was admitted to our hospital with symptoms of vomiting and epigastric pain. Abdominal computer tomography revealed a sizable formation that was diagnosed as a tumor of the pancreas. In laparotomy, a choloperitoneum was diagnosed as the cause of patient's symptoms. A tumor adherent firmly to the lesser curvature of stomach was also discovered. Cholecystectomy and subtotal gastrectomy were performed. Histologically, the tumor was diagnosed as a EGIST of the lesser omentum. The patient did not receive any adjuvant therapy and after two years of follow up she is without any recurrence.DISCUSSIONOmental EGISTs may remain clinically silent despite the large tumor's size. It is difficult to differentiate a EGIST in the lesser omentum from a GIST of the lesser curvature of the stomach, despite the use of advanced radiological imaging techniques. Our case of a giant EGIST of lesser omentum obscuring the diagnosis of acute choloperitoneum is the only one reported in literature.CONCLUSIONEGISTs that arise from the omentum are very rare and complete surgical resection is the only effective treatment approach. Adjuvant therapy following resection of localized disease has become standard of care in high risk cases.     

Solitary rectal cap polyp: Case report and review of the literature

Rectal bleeding combined with the presence of a rectal mass has been traditionally associated with the presence of malignant disease.Cap polyposis is a relatively young and still undefined rare entity which mainly involves the rectosigmoid.It is characterized by the presence of inflammatory polyps.In this case report,we present a patient who was diagnosed with a solitary cap polyp of the rectum during the investigation of a bleeding rectal mass.The patient’s age and the absence of family history were not in favor of malignancy,despite the strong initial clinical impression.After confirmation of the diagnosis,the patient underwent a snare excision and remains asymptomatic.Cap polyposis,although rare,should be suspected and,when diagnosed,should be treated according to location,number of polyps and severity of symptoms.     

Kikuchi’s lymphadenopathy: a relatively rare but important cause of lymphadenopathy in Greece, potentially associated with the antiphospholipid syndrome

Kikuchi-Fujimoto disease is a form of reactive lymphadenopathy, which was firstly described in Japan, but is uncommon in the Western world. We retrospectively reviewed the medical records of nine cases of adult or adolescent Kikuchi’s disease diagnosed in a single Haematology Unit in Athens, Greece between 1990 and 2006. The median age of the patients was 25 years (14–40) and 8/9 were females. All patients presented with cervical lymphadenopathy sparing the supraclavicular fossa; one had associated axillary lymphadenopathy, seven had fever and two were asymptomatic. The median duration of lymphadenopathy before presentation was 30 days (10–45). Just palpable splenomegaly was recorded in three patients. The median value of the maximal lymph node diameter was 2 cm (1–5) and only 1/9 had nodes >2 cm in their largest diameter. Lymphadenopathy was tender in two patients; hard nodes were observed in three patients. The median leukocyte count was 4.7 × 10⁹/l (2.2–4.9) with a normal differential in 7/9 patients. No infectious agent could be demonstrated. One patient had clinical and laboratory evidence of primary antiphospholipid syndrome (APLS). In conclusion, Kikuchi’s disease represents a rare but important diagnostic possibility for patients presenting with lymphadenopathy in Greece and other western countries. In this setting, autoimmune disorders, mainly lupus and APLS, should be considered and excluded by the appropriate laboratory work-up.     

Endothelin-B receptors and ventricular arrhythmogenesis in the rat model of acute myocardial infarction

The arrhythmogenic effects of endothelin-1 (ET-1) are mediated via ETA-receptors, but the role of ETB-receptors is unclear. We examined the pathophysiologic role of ETB-receptors on ventricular tachyarrhythmias (VT/VF) during myocardial infarction (MI). MI was induced by coronary ligation in two animal groups, namely in wild-type (n = 63) and in ETB-receptor-deficient (n = 61) rats. Using a telemetry recorder, VT/VF episodes were evaluated during phase I (the 1st hour) and phase II (2–24 h) post-MI, with and without prior β-blockade. Action potential duration at 90% repolarization (APD90) was measured from monophasic epicardial recordings and indices of sympathetic activation were assessed using fast-Fourier analysis of heart rate variability. Serum epinephrine and norepinephrine were measured with radioimmunoassay. MI size was similar in the two groups. There was a marked temporal variation in VT/VF duration; during phase I, it was higher (p = 0.0087) in ETB-deficient (1,519 ± 421 s) than in wild-type (190 ± 34 s) rats, but tended (p = 0.086) to be lower in ETB-deficient (4.2 ± 2.0 s) than in wild-type (27.7 ± 8.0 s) rats during phase II. Overall, the severity of VT/VF was greater in ETB-deficient rats, evidenced by higher (p = 0.0058) mortality (72.0% vs. 32.1%). There was a temporal variation in heart rate and in the ratio of low- to high-frequency spectra, being higher (<0.001) during phase I, but lower (p < 0.05) during phase II in ETB-deficient rats. Likewise, 1 h post-MI, serum epinephrine (p = 0.025) and norepinephrine (p < 0.0001) were higher in ETB-deficient (4.20 ± 0.54, 14.24 ± 1.39 ng/ml) than in wild-type (2.30 ± 0.59, 5.26 ± 0.67 ng/ml) rats, respectively. After β-blockade, VT/VF episodes and mortality were similar in the two groups. The ETB-receptor decreases sympathetic activation and arrhythmogenesis during the early phase of MI, but these effects diminish during evolving MI.     

Adolescents' wellbeing and functioning: relationships with parents' subjective general physical and mental health

Background  

This study aimed at examining the relationship between parental subjective health status and adolescents' health-related quality of life (HRQoL) as well as the role of gender, socioeconomic status, presence of chronic health care needs and social support on the above interaction.     

Risk assessment of diesel exhaust and lung cancer: combining human and animal studies after adjustment for biases in epidemiological studies

Background  

Risk assessment requires dose-response data for the evaluation of the relationship between exposure to an environmental stressor and the probability of developing an adverse health effect. Information from human studies is usually limited and additional results from animal studies are often needed for the assessment of risks in humans. Combination of risk estimates requires an assessment and correction of the important biases in the two types of studies. In this paper we aim to illustrate a quantitative approach to combining data from human and animal studies after adjusting for bias in human studies. For our purpose we use the example of the association between exposure to diesel exhaust and occurrence of lung cancer.