慢性中性粒细胞白血病伴意义未明单克隆免疫球蛋白增高1例并文献复习

目的:研究慢性中性粒细胞白血病(CNL)伴单克隆免疫球蛋白增高这种罕见血液病。方法:报告1例CNL伴单克隆免疫球蛋白增高患者,并复习国内外相关文献。结果:患者中性粒细胞明显增多,脾大,中性粒细胞碱性磷酸酶积分增高,无Ph染色体及BCR/ABL融合基因,骨髓粒系增生,无病态造血和明显纤维化依据,排除了如类白血病反应、其他克隆性血液病引起的中性粒细胞增多,合并单克隆免疫球蛋白增高意义未明,目前已排除淋巴瘤,尚不能诊断为多发性骨髓瘤。结论:CNL是一种少见的骨髓增殖性疾病,CNL与单克隆免疫球蛋白增高的相关性和治疗方法需要进一步明确。     

原发性血小板增多症患者治疗中出现慢性粒细胞白血病1例

目的认识Ph1阴性原发性血小板增多症在病程中向BCR-ABL1阳性慢性粒细胞白血病的转变。方法密切监测患者骨髓巨核细胞的形态、BCR/ABL P210融合基因转录水平、FISH法Ph1染色体、JAK2基因V617F突变。结果初诊时患者骨髓巨核细胞胞体大、多分叶核,BCR/ABL P210融合基因阴性,Ph1染色体阴性,JAK2基因为野生型;33个月后患者骨髓巨核细胞形态转为单圆核小巨核细胞,BCR/ABL P210融合基因转为阳性,Ph1染色体转为阳性,JAK2基因仍为野生型。结论该患者由Ph1阴性原发性血小板增多症在治疗过程中转为BCR-ABL1阳性慢性粒细胞白血病。     

不典型慢性粒细胞性白血病1例报道并文献复习

不典型慢性粒细胞白血病(atypical chronic myeloid leukaemia,aCML)是Ph染色体及bcr-abl融合基因均阴性的克隆性造血异常疾病,具有骨髓增生异常和骨髓增殖性肿瘤(MDS/MPN)的双重特性[1].其临床特点:骨髓以成熟中性粒细胞及中、晚幼粒细胞的增多,可伴有轻度贫血、血小板减少等类似慢性粒细胞白血病(chronic myeloid leukaemia,CML)的症状,但Ph染色体及bcr/abl融合基因均阴性.2008年WHO肿瘤分类中,将aCML归类MDS/MPN[2].本病的临床表现和生物学特性与慢性粒单核细胞白血病(chronic myelomonocytic leukaemia,CMML)、慢性中性粒细胞白血病(chronic neutrophilic leukemia,CNL)等有许多相似之处,这给诊断带来一定困难,本文报道1例确诊的aCML.     

慢性中性粒细胞白血病5例临床分析

目的：探讨慢性中性粒细胞白血病（CNL）的诊断，治疗和预后。方法：对5例CNL患者的临床表现，实验室特点，治疗方法和预后进行分析。结果：5例CNL患者中，4例脾脏肿大；所有患者血液中中性粒细胞持续增多，骨髓粒系增生并以成熟中性粒细胞为主，中性粒细胞碱性磷酸酶（NAP）积分增高，Ph染色体或bcr/abl融合基因阴性；使用马利兰及羟基脲治疗后患者症状有所改善，脾脏缩小及血液中白细胞数下降；1例患者诊断后7年发生急性变。结论：CNL是一种少见类型的慢性白血病，其特点与慢性髓细胞白血病不同；马利兰及羟基脲对其有一定疗效；预后具有异质性。     

BCR／ABL基因与白血病

BCR／ABL融合基因几乎见于所有的慢性粒细胞性白血病（CML）及25％～50％的急性B系淋巴细胞性白血病（ALL）和约5％的急性粒细胞性白血病（AML）中“’。包括转染及转基因鼠在内的大量实验资料表明，BCR／ABL融合基因在上述白血病的发病中起重要作用。本文就目前的研究状况从①BCR／ABL基因的分子生物学特性；＠BCR／ABL基因引起肿瘤转化的可能机制；③BCR／ABL基因在引起复杂白血病类型中的特殊作用，对BCR／ABL基因在白血病发生发展中的作用作一综述。IBCR／ABL基因的分子生物学特性BCR／ABL基因是由9号与22号染…     

巢式聚合酶链反应检测慢性粒细胞白血病bcr／abl融合基因

目的 探讨Ph染色体和bcr/abl融合基因在慢性粒细胞白血病（CGL）的发病机制、诊断、治疗、预后判断的价值。方法 对46例CGL患者作巢式逆转录聚合酶链反应（RT-PCR）检测bcr/abl融合基因,同时对其中28例作细胞遗传学检查。结果 46例CGL患者中,44例bcr/abl融合基因阳性,阳性率为95.7%;28例CGL患者作细胞遗传学检查。26例Ph染色体阳性,阳性率为92.9%;2例Ph染色体阴性的CGL患者,用RT-PCR检测出ber/abl融合基因。结论 巢式RT-PCR是一种快速、敏感而准确的检测方法,可以为部分Ph染色体阴性的CGL患者提供分子生物学的诊断依据。     

甲磺酸伊马替尼治疗进展期慢性髓细胞白血病14例疗效分析

慢性髓细胞白血病(CML)是起源于造血干细胞的恶性克隆性疾病,由于9号染色体的ABL基因与22号染色体的BCR基因相互易位形成BCR/ABL融合基因,表达P210蛋白,具有异常的酪氨酸激酶活性,激活细胞信号传导途径,促进细胞增殖,抑制细胞凋亡.     

荧光原位杂交检测慢性粒细胞白血病衍生9号染色体缺失的研究

目的:运用特殊位点(LSI)9q34探针荧光原位杂交技术(FISH)研究慢性粒细胞白血病(CML)患者衍生9号染色体部分序列缺失的情况,并探讨临床意义。方法:对42例CML患者进行常规染色体核型分析,采用BCR/ABL双色双融合或ES探针对骨髓间期细胞进行FISH检测,同时运用9q34探针检测有无衍生9号染色体部分序列缺失。结果:42例染色体核型分析中,35例为典型的t(9;22)染色体易位,4例为复杂易位。42例患者均行FISH检测证实为BCR/ABL融合基因阳性。运用9q34探针检测所有42例患者中发现有6例伴有衍生der(9)部分序列缺失,发生率为14%,发生复杂易位的4例患者均未发现der(9)部分缺失。6例发生缺失的患者仅有1例处于慢性期,其中3例患者病程仅为1-2个月,初诊就已诊断为CML加速期或急变期。结论:在CML患者中der(9)部分缺失的发生率为14%,伴有der(9)部分缺失的患者多表现为慢性期短,建议所有BCR/ABL融合基因阳性的CML患者应常规使用9q34探针检测der(9)有无缺失。     

实时定量PCR检测慢性粒细胞白血病微小残留病研究进展

慢性粒细胞白血病(CML)是起源于造血干细胞的恶性克隆性疾病。约90%～95%CML患者具有费城染色体(Philadelphia,Ph),其分子基础是9号染色体上的c-abl基因易位到22号染色体上的bcr基因上形成新的基因嵌合体-bcr/abl融合基因,即t(9;22)(q34;q11)。该基因表达P210bcr/abl融合蛋白具     

Three layer functional model and energy exchange concept of aging process

Relying on a certain degree of abstraction, we can propose that no particular distinction exists between animate or living matter and inanimate matter. While focusing attention on some specifics, the dividing line between the two can be drawn. The most apparent distinction is in the level of structural and functional organization with the dissimilar streams of ‘energy flow’ between the observed entity and the surrounding environment. In essence, living matter is created from inanimate matter which is organized to contain internal intense energy processes and maintain lower intensity energy exchange processes with the environment. Taking internal and external energy processes into account, we contend in this paper that living matter can be referred to as matter of dissipative structure, with this structure assumed to be a common quality of all living creatures and living matter in general. Interruption of internal energy conversion processes and terminating the controlled energy exchange with the environment leads to degeneration of dissipative structure and reduction of the same to inanimate matter, (gas, liquid and/or solid inanimate substances), and ultimately what can be called ‘death.’ This concept of what we call dissipative nature can be extended from living organisms to social groups of animals, to mankind. An analogy based on the organization of matter provides a basis for a functional model of living entities. The models relies on the parallels among the three central structures of any cell (nucleus, cytoplasm and outer membrane) and the human body (central organs, body fluids along with the connective tissues, and external skin integument). This three-part structural organization may be observed almost universally in nature. It can be observed from the atomic structure to the planetary and intergalactic organizations. This similarity is corroborated by the membrane theory applied to living organisms. According to the energy nature of living matter and the proposed functional model, the decreased integrity of a human body's external envelope membrane is a first cause of the structural degradation and aging of the entire organism. The aging process than progresses externally to internally, as in single cell organisms, suggesting that much of the efforts towards the restoration and maintenance of the mechanisms responsible for structural development should be focused accordingly, on the membrane, i.e., the skin. Numerous reports indicate that all parts of the human body, like: bones, blood with blood vessels, muscles, skin, and so on, have some ability for restoration. Therefore, actual revival of not only aging tissue of the human body's membrane, but the entire human body enclosed within, with all internal organs, might be expected. We assess several aging theories within the context of our model and provide suggestions on how to activate the body's own anti-aging mechanisms and increase longevity. This paper presents some analogies and some distinctions that exist between the living dissipative structure matter and inanimate matter, discusses the aging process and proposes certain aging reversal solutions.     

Unusual responses of nocturnal pineal melatonin synthesis and secretion to swimming: Attempts to define mechanisms

Abstract: The effect of swimming at night on rat pineal melatonin synthesis was compared with that of light exposure at night. Rats were forced to swim at 0030 hr (lights out at 2000 hr) and sacrificed by decapitation 15 and 30 min later, immediately after swimming. Other groups of animals were exposed to white light (650μW/cm²) for 15 and 30 min at same time. Swimming caused a rapid and highly significant drop in the melatonin content in the pineal gland; however, the activity of N-acetyltransferase (NAT), the supposed rate limiting enzyme in the melatonin production, was not changed. Despite the drop in pineal melatonin levels, serum concentrations of the indole remained elevated in the rats that swam. In contrast, melatonin levels in the pineal and serum of light exposed rats fell precipitously, accompanied by a significant suppression of NAT activity. Since we anticipated that the strenuous exercise associated with swimming may induce release of artrial natriuretic peptide (ANP) from the heart, which in turn could cause the release of pineal melatonin, in a second study we injected physiological saline intravenously to stretch the cardiac muscle and release ANP. Three milliliters of normal saline was injected during the day into the jugular vein of anesthetized rats that were pretreated with isoproterenol to stimulate pineal melatonin production. Animals were killed 15 min after the saline injection, and pineal NAT activity and pineal melatonin levels were measured. The saline injections caused no alteration in the elevated levels of either NAT or melatonin. These data suggest that the disparity in pineal NAT activity (which was high) and pineal melatonin (which was low), in animals swum at night, may not be caused by ANP which is released during strenuous exercise such as swimming.     

Melatonin and photoperiodic time measurement: Seasonal breeding in the sheep

Abstract: Well-established circadian physiology supports the view that photoperiodic time measurement utilizes the coincidence between the presence of light and a photosensitive phase of a 'biological clock' to alter reproductive status—the so-called external coincidence model of seasonal breeding. In this review, we examine the mechanism whereby photoperiod interacts with presumed suprachiasmatic nuclei activity to allow endogenous melatonin to normally synchronize reproductive activity to the optimal time of year. The Romney Marsh sheep is particularly explored as an experimental model. It is suggested that the on/off activity of seasonal reproduction may be a robust mechanism able to be predictably manipulated by the judicious use of the light/dark cycle and exogenous melatonin, but firmly based on circadian principles.     

The immunoneuroendocrine role of melatonin

Abstract: A tight, physiological link between the pineal gland and the immune system is emerging from a series of experimental studies. This link might reflect the evolutionary connection between self-recognition and reproduction. Pinealectomy or other experimental methods which inhibit melatonin synthesis and secretion induce a state of immunodepression which is counteracted by melatonin. In general, melatonin seems to have an immunoenhancing effect that is particularly apparent in immunodepressive states. The negative effect of acute stress or immunosuppressive pharmacological treatments on various immune parameters are counteracted by melatonin. It seems important to note that one of the main targets of melatonin is the thymus, i.e., the central organ of the immune system. The clinical use of melatonin as an immunotherapeutic agent seems promising in primary and secondary immunodeficiencies as well as in cancer immunotherapy. The immunoenhancing action of melatonin seems to be mediated by T-helper cell-derived opioid peptides as well as by lymphokines and, perhaps, by pituitary hormones. Melatonin-induced-immuno-opioids (MHO) and lymphokines imply the presence of specific binding sites or melatonin receptors on cells of the immune system. On the other hand, lymphokines such as -γ-interferon and interleukin-2 as well as thymic hormones can modulate the synthesis of melatonin in the pineal gland. The pineal gland might thus be viewed as the crux of a sophisticated immunoneuroendocrine network which functions as an unconscious, diffuse sensory organ.     

Response of rat pineal melatonin to calcium, magnesium, and lithium is circadian stage dependent

Abstract: Herein we documented the response of pineal melatonin production to electrolytes known to be effective on pineal function in view of a possible circadian stage dependence. We studied the release of melatonin by perifused rat pineal glands at 2 different circadian stages corresponding to the middle of the light and dark periods, i.e., respectively, 7 and 19 HALO (Hours After Light Onset, L:D = 12:12). The initial efflux rates were, as expected, much higher in the perifusates of glands removed from rats sacrificed during the dark phase than of those removed during the light phase. After 3 hr of perifusion, melatonin release reached similar levels which were found constant up to the 8th hr of perifusion, whatever the circadian stage. Perifusion of the glands with physiological concentrations for the rat of calcium (5.2 mmol/1) and magnesium (1.34 mmol/1) resulted in a stimulatory effect on the pineal glands removed from rats sacrificed in the middle of the dark period (19 HALO), whereas no effects were observed on the pineal glands removed from rats sacrificed during the light (7 HALO). Lithium (0.28 and 0.55 mmol/1) was ineffective on melatonin release in pineal glands removed 7 and 19 HALO. Our results show differences in the initial efflux rates of melatonin and in the response of perifused pineal glands to calcium and magnesium according to the circadian stage.     

Serological survey of HIV and syphilis in pregnant women in Madagascar

Objectives Peripartal transmission of human immunodeficiency virus (HIV) and Treponema pallidum, the causative agent of syphilis, leads to severe consequences for newborns. Preventive measures require awareness of the maternal infection. Although HIV and syphilis testing in Madagascar could be theoretically carried out within the framework of the national pregnancy follow‐up scheme, the required test kits are rarely available at peripheral health centres. In this study, we screened blood samples of pregnant Madagascan women for HIV and syphilis seroprevalence to estimate the demand for systemic screening in pregnancy. Methods Retrospective anonymous serological analysis for HIV and syphilis was performed in plasma samples from 1232 pregnant women that were taken between May and July 2010 in Ambositra, Ifanadiana, Manakara, Mananjary, Moramanga and Tsiroanomandidy (Madagascar) during pregnancy follow‐up. Screening was based on Treponema pallidum haemagglutination tests for syphilis and rapid tests for HIV, with confirmation of positive screening results on line assays. Results Out of 1232 pregnant women, none were seropositive for HIV and 37 (3%) were seropositive for Treponema pallidum. Conclusions Our findings are in line with previous studies that describe considerable syphilis prevalence in the rural Madagascan population. The results suggest a need for screening to prevent peripartal Treponema pallidum transmission, while HIV is still rare. If they are known, Treponema pallidum infections can be easily, safely and inexpensively treated even in pregnancy to reduce the risk of transmission.     

Conservative management of perforated duodenal diverticulum： A case report and review of the literature

Duodenal diverticula are a relatively common condition. They are asymptomatic, unless they become complicated, with perforation being the rarest but most severe complication. Surgical treatment is the most frequently performed approach. We report the case of a patient with a perforated duodenal diverticulum, which was diagnosed early and treated conservatively with antibiotics and percutaneous drainage of secondary retroperitoneal abscesses. We suggest this method could be an acceptable option for the management of similar cases, provided that the patient is in good general condition and without septic signs.