2009年美国血液病学年会造血干细胞移植热门临床研究报告

<正>2009年美国血液病学年会上研究者对异基因造血干细胞移植(allogeneic hematopoietic stem cell transplantation,allo-HSCT)予以极大的关注,相关会议摘要超过1200篇。本文就一些涉及重要的、被反     

Delayed diagnosis of Gorlin's syndrome in a renal transplant recipient

A 35-year-old woman was referred to the dedicated dermatology clinic for RTR. She underwent her first renal transplant at 18 years of age due to chronic renal failure following reflux nephropathy of the single right kidney (left kidney agenesis). She has since then had two further transplants. During clinical examination she was noted to have at least 16 basal cell carcinomas (BCC) and there are records in the case notes of 10 BCC having being excised and confirmed histologically in the past. By contrast, she had only had two squamous cell carcinomas (SCC) excised. She was also noted to have distinctive facial features, a kyphoscoliosis and palmar pits, and a diagnosis of Gorlin's (naevoid BCC) syndrome (GS) was made. Although immunosuppression may have contributed to the multiplicity of her BCC, the contrasting very small number of SCC is unusual in an organ transplant recipient and this alerted the authors to the diagnosis of GS.     

Doppler-guided haemorrhoidal artery ligation: long-term outcome and patient satisfaction

Objective  Conventional Milligan–Morgan haemorrhoidectomy is associated with significant pain and potentially hazardous complications. Doppler-Guided Haemorrhoidal Artery Ligation (DGHAL) may offer a lower risk, pain-free alternative. We present our early and long-term outcome experience with DGHAL, combined with patient views and satisfaction with the procedure.
Method  One hundred and thirteen DGHALs were performed over a 13 month period by two surgeons in a single centre. Patients graded the severity of postoperative pain on visual-analogue scales. Clinical follow-up was at 6 weeks ( n  = 103), with long-term follow-up ( n  = 90) by postal questionnaire at median of 30 months.
Results  Seven out of one hundred and three (6%) patients reported postoperative discomfort requiring analgesia. Ninety-three out of one hundred and three (90%) patients reported complete relief or significant improvement in their symptoms at 6 weeks, dropping to 77/90 (86%) at 30 months. Anal fissures developed in 2/103 (2%) patients, both treated with Diltiazem ointment. Further surgery was required in 8/90 (9%) patients. Eighty-two out of ninety (91%) patients said they would undergo DGHAL again.
Conclusion  DGHAL is a relatively painless, safe, and effective procedure for symptomatic stage I–III haemorrhoids, for which we have demonstrated long-term durability and acceptability. Its role lies between office based procedures and more invasive operative interventions.     

克拉屈滨治疗巨球蛋白血症后早期发生自身免疫性溶血性贫血

Waldenstr¨oms巨球蛋白血症(WM)是一种罕见的惰性B细胞淋巴瘤,其特点为骨髓中有浆细胞样淋巴细胞浸润,常伴有单克隆IgM副蛋白。IgM副蛋白引起高粘滞血症、冷凝集素性溶血性贫血、冷球蛋白血症、多发性神经病。WM的这些典型特征通常表现出症状。WM对化疗有反应、多次复发和生存     

Personality traits and psychological reactions to mental stress of female migraine patients

The purpose of this study was (i) to compare a range of stress-related personality traits, including defense and coping mechanisms, of migraine patients (n = 23) with those of tension headache patients (n = 18) and dermatologically afflicted, but otherwise healthy, controls (n = 22), and (ii) to compare their state anxiety and other moods before, during, and after the presentation of a psychological stressor (mental arithmetic). For all three groups, mental arithmetic induced a significant increase in state anxiety and mood disturbance, followed by a subsequent decrease during recovery. Migraine patients were not found to have a higher disposition for anxiety, depression, or rigidity than tension headache patients or controls. Between the headache groups no differences in the use of defense and coping mechanisms were found. Compared to the control group, however, both migraine patients and tension headache patients were more inclined to use internally focused defense mechanisms and less inclined to seek social support when confronted with a problem. The psychological reaction of migraine patients to mental stress hardly differed from tension headache and control subjects. Compared to the control subjects, however, both groups of headache patients exhibited a diminished recovery from feelings of vigour, depression, and fatigue due to the stress induced. It is suggested that this distinct psychological reaction to stress of headache patients versus healthy control subjects is related to the more internally focused defense style of the headache sufferers. Thus, in contrast to previous results, this study does not present evidence of a migraine personality. It suggests the development of specific personality characteristics as a consequence of suffering from episodic headache.     

Serum leptin during recovery following maximal incremental and prolonged exercise.

This study investigated the delayed circulating leptin response to maximal and prolonged treadmill exercise. Six healthy untrained males performed three sessions after an overnight fast: control, maximal exercise, and prolonged exercise at 50% of maximal oxygen consumption. Blood samples were collected prior to exercise, at the end of exercise, and at 60, 120, 180, and 240 min following exercise and control sessions. Blood samples were analyzed for serum leptin, insulin, glucose, free fatty acids, and glycerol. Hemoglobin and hematocrit were measured to correct for plasma volume changes. Resting energy expenditure (REE) and body fat (BF) were also assessed. Immediately at the end of maximal and prolonged exercise, and during the 4 hours of recovery, serum leptin levels did not change significantly compared to their respective baseline values. At 240 min of recovery serum leptin decreased 7% and 9% (p>0.05) from the baseline in the maximal and prolonged sessions, respectively. In the control experiment serum leptin decreased 27% from the baseline at 240 min of the recovery (p < 0.05). No significant differences were found in leptin values between the control and exercise sessions. Control serum leptin was positively correlated (p < 0.05) to BF (r = 0.88) and glucose (r=0.96), and negatively correlated to REE (r= -0.81). In conclusion, maximal or prolonged exercise do not appear to have an influence on circulating serum leptin in the delayed (4 hr) post exercise recovery period.     

Anopheles (Anopheles) forattinii: a new species in Series Arribalzagia (Diptera: Culicidae).

Anopheles (Anopheles) forattinii new species, a member of the Series Arribalzagia, is described with illustrations of the larval and pupal stages, and male and female genitalia. It is contrasted with 2 similar species, An. (Anopheles) costai Fonseca & Ramos and An. (Anopheles) mediopunctatus (Lutz). This species, and An. costai, occur over much of South America where both have been misidentified as An. mediopunctatus, a species presently only known from southeastern Brazil.