血小板异常并发脑卒中临床分析

目的:总结血小板异常患者并发脑卒中的临床特点.方法:回顾性分析1999年1月至2009年1月中山大学孙逸仙纪念医院收治的血小板异常(原发性血小板增多症、特发性血小板减少性紫癜)并发脑卒中患者的临床资料.结果:原发性血小板增多症(ET)患者60例中,10%(6/60)出现脑梗死,年龄为69±11岁.血小板计数为(1112±366)×109/L;1.7e(1/60)出现脑出血,54岁,血小板计数为597×109/L.特发性血小板减少性紫癜(ITP)患者696例中,0.6%(4/696)患者出现脑出血,年龄为56±30岁,血小板计数为(29±12)×109/L:ITP患者中0.3%(2/696)出现脑梗死,分别为64岁、73岁,血小板计数分别为49×109/L、166×109/L.结论:ET患者脑梗死发生率高,建议二级预防治疗中强化抗血小板治疗以预防再次脑梗塞;ITP患者并发卒中少见,二级治疗中建议谨慎选择抗血小板方案.     

MDS/MPN伴环形铁粒幼细胞和血小板增多2例并文献复习

目的探讨骨髓增生异常/骨髓增殖性肿瘤伴环形铁粒幼细胞和血小板增多(MDS/MPN-RS-T)的临床特点及诊断标准。方法对2例确诊MDS/MPN-RS-T患者进行回顾性分析并文献复习。结果 2例患者均有贫血,骨髓环形铁粒幼细胞大于15%,PLT持续增高,未曾诊断过MDS伴环形铁粒幼细胞(MDS-RS)或原发性血小板增多症(ET),JAK2V617F基因突变阳性,1例患者SF3B1阳性,BCR-ABL1、MPL、CALR等基因均阴性,符合MDS/MPN-RS-T的诊断。结论贫血、PLT增高及环形铁粒幼细胞增多要高度怀疑MDS/MPN-RS-T,细胞形态学、细胞化学及细胞遗传学检查三者联合检查有助于诊断及鉴别诊断。     

人物介绍

原发性血小板增多症(essential throbocythemia,ET)是一种慢性骨髓增殖性疾病,以血小板持续增高为特征,临床上主要表现为出血或血栓症状[1].我们对2例ET患者血栓止血相关检测项目进行分析,以评估其检测结果在患者诊断、治疗中的意义. 一、病例 1.病例1 男,82岁,因“白细胞升高3年,左胸部肿块1周,皮肤瘀斑3d”来襄阳医院就诊,门诊查白细胞计数(37.64 × 109/L)、血小板计数(1 734×109/L)高,遂以“血小板增多原因待查”收入院.     

具有JAK2V617F突变的原发性血小板增多症转化为急性髓系白血病1例并文献复习

患者,男,53岁,因发现脾大(B超12.6 cm×5.0 cm)7个月于2005年4月8日就诊于我科.查体未发现异常.血常规:白细胞(WBC)10.5×109/L,血红蛋白(Hb)149 g/L,血小板(Plt)1 314×109/L;骨髓象:有核细胞增生活跃,粒红两系正常,巨核细胞63只,血小板成堆可见;BCR/ABL融合基因阴性,诊断为原发性血小板增多症(ET).     

原发性血小板增多症致假性高钾血症1例并文献复习

正原发性血小板增多症是骨髓增殖性疾病的一种,其特征是血小板水平显著持续性增多伴功能异常。本文报道我院诊断的一例原发性血小板增多症致假性高钾血症并结合文献复习假性高钾血症发生的原因。1临床资料病历摘要:患者女,64岁,主因"间断全身乏力1月余"于2016年2月9日入院。1月余前无诱因出现上腹胀痛,伴全身酸痛、乏力,就诊于当地医院,化验血常规:白细胞12.3×10~9/L,血红蛋白96g/L,血小板1464×10~9/L,行骨髓穿刺示:骨髓粒系、红系、巨核系增生活跃,血小板聚集成片,散在多见。诊断为"原发性血小板增多症",当地医院给予羟基脲     

血小板单采治疗原发性血小板增多症的疗效及护理

目的:探讨血小板单采治疗原发性血小板增多症的疗效及护理要点。方法:对22例次血小板单采术治疗的原发性血小板增多症进行护理及观察,并检测治疗前后患者血小板计数。结果:血小板单采治疗前患者平均血小板水平为(1398±217)×109/L,单采治疗后为(936±185)×109/L,下降约1/3,两者相比差异有显著性(P<0.01);单采治疗护理要点为做好术前准备,术中密切观察患者的生命体征,及时发现不良反应先兆,严格遵守操作规程,保证良好的循环压力和流速,术后应观察出血情况。结论:血小板单采术能迅速、有效地降低患者血小板水平,改善临床症状。     

血小板低下经部分性脾动脉栓塞术后化疗的骨髓耐受性评价

目的评价恶性肿瘤患者通过部分性脾动脉栓塞术（partial splenic embolization,PSE）升高血小板后化疗的骨髓功能耐受性。方法本研究26例均为需要化疗的非肝癌恶性肿瘤合并血小板减少症患者,其中20例为病毒性肝炎后肝硬化门静脉高压症引起脾功能亢进性血小板减少症患者,6例为原发性血小板减少症患者。26例患者介入前血小板数量在（12～48）×109/L,平均（32.6±9.5）×109/L,其中11例白细胞在（2.8～3.5）×109/L。PSE后2周内血小板升到80×109/L以上,随后给予静脉联合化疗,每周期化疗后第3、7、10、14天复查血常规,并观察其他不良反应。结果PSE后2周内血小板最高值在（83～341）×109/L,平均（170.7±81.5）×109/L,较介入前差异有统计学意义（t=-8.197,P〈0.05）。26例患者共接受化疗127个周期,平均每例4.9个周期（3～6个周期）。不良反应主要表现为骨髓抑制和恶心、呕吐,无肝、肾功能损害。Ⅲ、Ⅳ度白细胞抑制率为30.8%（8/26）,Ⅲ、Ⅳ度血小板抑制率为11.5%（3/26）;恶心、呕吐发生率为61.5%（16/26）,Ⅲ度发生率为7.7%（2/26）,无Ⅳ度恶心、呕吐发生。结论对于肝硬化门静脉高压引起的脾功能亢进性血小板减少症和原发性血小板减少症恶性肿瘤患者,采用PSE升高血小板后进行化疗时,骨髓抑制可以耐受。     

血小板计数增多原因分析

血小板是血液检查的一个动态指标,也是血细胞计数中最困难的一项,血小板计数的正常参考范围为（100～300）×109/L.临床上血小板计数〉300×109/L的患者越来越多,常见原因多为原发性血小板增多症和继发性血小板增多两种情况.     

骨髓活检塑料包埋切片铁染色在血液病病理诊断中的意义

目的　研究铁染色在血液病骨髓活检病理诊断中的意义。方法　骨髓活检塑料包埋 ,半薄切片 ,Perls蓝染色 ,半定量分析。结果　 584例血液病中铁染色阳性率最低者为缺铁性贫血 (0 / 7) ,此后依次为特发性血小板减少性紫癜 (6 5 % ,4/ 62 ) ,真性红细胞增多症 (9 1 % ,1 / 1 1 ) ;阳性率较高者依次为纯红细胞再生障碍性贫血 (1 0 0 % ,8/ 8) ,骨髓增生异常综合征 (75 5 % ,40 / 53) ,巨幼细胞性贫血 (60 % ,9/ 1 5)。骨髓增生异常综合征铁染色阳性率显著高于慢性再生障碍性贫血和特发性血小板减少性紫癜 (P <0 0 1 ) ,纯红细胞再生障碍性贫血铁染色阳性率显著高于慢性粒细胞白血病 (P <0 0 1 ) ,骨髓增生异常综合征铁染色阳性率显著高于骨髓增殖性疾病 (不能分类 ) (P <0 0 1 )。结论　骨髓活检铁染色在某些血液病骨髓病理诊断及鉴别诊断中具有重要参考价值     

JAK2V617F点突变与BCR-ABL阴性骨髓增殖性疾病临床关系研究

目的 研究JAK2V617F点突变与BCR-ABL阴性的骨髓增殖性疾病(MPD)患者临床特征的关系.方法 选择62例BCR-ABL阴性MPD患者为研究组,包括真性红细胞增多症(PV)26例、原发性血小板增多症(ET)26例、原发性骨髓纤维化(IMF)9例、慢性中性粒细胞白血病(CNL)1例;同时选择慢性粒细胞白血病(CML)20例、急性白血病(AL)10例、健康志愿者15例为对照组.用等位基因特异性聚合酶链反应(AS-PCR)方法 结合基因测序检测各组患者JAK2V617F的突变情况,分析JAK2V617F点突变与BCR-ABL阴性的MPD患者临床特征的关系.结果62例BCR-ABL阴性的MPD患者中,44例JAK2V617F突变阳性,其中PV 23例(88.5%,23/26),ET 15倒(57.7%,15/26),IMF 5例(55.6%,5/9),CNL 1例;对照组均阴性.JAK2V617F突变阳性的MPD患者和突变阴性的MPD相比较,两组在发病年龄方面差异无统计学意义;在血象方面,两组PV患者的白细胞[(18.2±8.0)×109/Lvs(7.6±1.4)×109/L,P=0.035]、血小板计数[(479±141)×109/L vs(277±102)×109/L,P=0.025]及两组ET患者的血红蛋白[(146±16)g/L vs(122±17)g/L,P=0.001]、白细胞计数[(14.6±5.1)× 109/L vs(10.9±3.4)×109/L,P=0.044]差异均有统计学意义.另外,与JAK2V617F突变阴性的ET相比,突变阳性的ET患者出血,血栓、转白等并发症的发生率高(P=0.034).结论 应用AS-PCR法筛选JAK2V617F突变准确率高,可在临床上推广使用.与JAK2V617F突变阴性的ET患者相比较,突变阳性的ET患者的临床过程更具有侵袭性.     

Serum iron concentration as a tool to measure relative iron absorption from elemental iron powders in man

Objective: To present a new method for measuring the relative bioavailability (RBV) of commercial elemental iron powders by investigating induced changes of serum iron concentration (S‐Fe) in relation to ferrous sulphate (FeSO₄). Earlier studies have shown that in a group of subjects there is good agreement between the increase in S‐Fe and the amount of iron absorbed from a simple iron salt as FeSO₄. Methods: The study included two groups of male blood donors (n=2×16 subjects) who were served three meals with an interval of approximately nine weeks between each one. In one group the meal was fortified with reduced iron, ferrous sulphate or no iron at all. In the other group the meal was fortified with electrolytic iron, ferrous sulphate or no iron. The S‐Fe increase was followed for 6?h. Studying the non‐iron meals was necessary so that the basal diurnal variation in the S‐Fe during the six hours could be measured and subtracted. Results: The mean S‐Fe increase calculated as the area under the curve (AUC) from the reduced iron (RBV=0.43) and the electrolytic iron (RBV=0.73) differed significantly from the AUC following FeSO₄ (p=0.002 and p=0.021, respectively). The difference between the reduced and the electrolytic iron was also statistically significant (p=0.036). Conclusion: Measuring increases in S‐Fe could be a reliable and simple method to determine the RBV in comparative studies of elemental iron powders in relation to FeSO₄.     

骨髓铁与常用血清铁指标的相关性研究

目的 探讨血清铁(SI)、血清铁蛋白(SF)、总铁结合力(TIBC)、不饱和铁结合能力(UIBC)、转铁蛋白(TRF)等血清铁指标和骨髓内、外铁的相关性.方法 对112例贫血患者进行骨髓铁染色及血清常用铁指标的检测.将骨髓内、外铁与常用铁指标进行等级相关统计分析.按骨髓内、外铁变化的情况将病例分组:骨髓内、外铁均下降为...     

Thrombocytopenia secondary to iron deficiency anemia responding to iron therapy

A broad spectrum of diseases can cause anemia and thrombocytopenia. Some of these diseases are a hematological emergency; others are benign diseases, so early and accurate diagnosis is crucial in managing such patients. Usually, IDA is associated with thrombocytosis or normal platelets; however, in rare cases, IDA can be associated with thrombocytopenia; even though, thrombocytopenia that occurs with IDA responds to iron therapy. Iron therapy rarely causes transient thrombocytopenia per se. We are reporting an African female patient who is found to have thrombocytopenia secondary to iron deficiency anemia (IDA), and she responded to iron replacement therapy initially with a transient drop in platelets, followed by a rapid rise in platelets till platelets reached the normal level.     

Non-transferrin-bound iron is present in serum of hereditary haemochromatosis heterozygotes

BACKGROUND: Hereditary haemochromatosis (HH) is a common autosomal recessive disease. Recently, HH heterozygosity has been identified as an independent risk factor for myocardial infarction and cardiovascular mortality. Iron may play an important role in atherogenesis by catalyzing peroxidation of low-density-lipoprotein (LDL), an essential step in atherogenesis. In iron overload conditions, non-transferrin-bound iron (NTBI) is found in serum, which can catalyze lipid peroxidation. We investigated whether sera of HH heterozygotes contain more NTBI than sera of normal controls. METHODS: In 27 treated HH homozygotes, 22 HH heterozygotes and 17 healthy control subjects, conventional parameters of iron status (serum iron, transferrin saturation, serum ferritin) were measured. NTBI was detected using HPLC after addition of nitrilotriacetic acid and pretreatment with cobalt. RESULTS: The conventional parameters of iron status were similar in the HH heterozygous group and the control group. NTBI was significantly higher in homozygotes compared to heterozygotes (1.79 micromol L-1 vs. 0.51 micromol L-1, 95% CI of the difference = 0.6-1.95, P < 0.001), and controls (1.79 micromol L-1 vs. - 0.3 micromol L-1, 95% CI of the difference = 1.36-2.81, P < 0.001). The difference in NTBI between the heterozygous subjects and control subjects was also significant (0.51 micromol L-1 vs. - 0. 3 micromol L-1, 95% CI of the difference = 0.05-1.57, P < 0.05). CONCLUSION: Phlebotomy treated HH homozygotes maintain a high and potentially harmful serum NTBI. HH heterozygotes have a higher serum NTBI than normal controls. The reported increased risk of cardiovascular events in heterozygous haemochromatosis may be explained by NTBI-catalyzed LDL peroxidation.     

维生素AD联合铁剂治疗婴幼儿缺铁性贫血的效果

目的:观察维生素AD滴剂联合蛋白琥珀酸铁口服液治疗婴幼儿缺铁性贫血的临床效果。方法:选取安徽省庐江县人民医院儿童保健门诊缺铁性贫血患儿102例,性别不限,采用随机数字表法分为观察组(n=52)和对照组(n=50)。观察组治疗采用蛋白琥珀酸铁口服液联合维生素AD滴剂口服,对照组治疗仅采用蛋白琥珀酸铁口服液口服。治疗前分析缺铁性贫血患儿维生素A和25-羟维生素D缺乏情况,于治疗1个月后对2组治疗效果进行比较并对贫血相关指标进行分析。结果:治疗前两组缺铁性贫血患儿维生素A和25-羟维生素D缺乏严重。治疗后,观察组的显效率为61.54%,明显高于对照组的44%(P<0.05);总有效率为94.23%,也明显高于对照组的84%(P<0.05);观察组血红蛋白、血清铁和血清铁蛋白较对照组明显改善(P<0.05)。结论:维生素AD滴剂联合蛋白琥珀酸铁口服液用于婴幼儿缺铁性贫血治疗时可有效改善患儿相关的贫血指标,显著提高临床治疗效果。     

Non-haem iron transport in the rat proximal colon

BACKGROUND: Only 10% of dietary iron is absorbed in the duodenum which implies that 90% (approximately 9 mg day(-1)) reaches the lower small intestine and colon. Therefore the purpose of this study was to assess the iron transport capacity of the rat proximal colon and to determine whether iron absorption is regulated by changes in dietary iron content. MATERIALS AND METHODS: Rats were fed for 14 days on either iron adequate (44 mg Fe kg(-1) diet) or iron-deficient (< 0.5 mg Fe kg(-1) diet) diets. The 59Fe transport across the colonic epithelium and its subsequent appearance in the blood were measured in vivo. In separate studies the colon was excised and used to measure divalent metal transporter expression. RESULTS: Divalent metal transporter (DMT1) was expressed at the apical membrane of the surface epithelium in rat proximal colon. In animals fed an iron-deficient diet, DMT1 mRNA and protein expression were increased. This was accompanied by a significant increase in tissue 59Fe uptake. CONCLUSIONS: The proximal colon can absorb non-haem iron from the intestinal lumen. The purpose of this mechanism remains to be elucidated.     

The iron hypothesis of atherosclerosis and its clinical impact

The iron hypothesis as an alternative explanation for the gender difference in the incidence and mortality of atherosclerosis has provoked increased debates and public health concerns. In this review we summarize the historical and recent literature on the iron hypothesis and discuss several related clinical issues and their implications. Apart from misconstruction of study populations, lack of a good method to reflect the iron contents of tissues may be the major factor for causing inconsistent results from epidemi‐ological studies. Published data from 11 countries clearly indicate that the mortality from cardiovascular diseases is correlated with liver iron. We propose that redox‐active iron in tissue is the atherogenic portion of total iron stores. Recently developed magnetic resonance imaging techniques in combination with Fe chelators may allow future studies to examine this component of body iron in lesions and the whole body. Several clinical situations characterized by increased iron stores have been proposed as ‘human models’ suitable for further tests of the iron hypothesis. Patients with end‐stage renal disease may be the most unique cohort, having significant increases in their iron stores, low‐density lipoprotein (LDL) oxidation, and cardiovascular events. Other patient groups may be well suited for specific studies of different atherogenic events. With a better understanding of iron‐driven oxidative damage, well controlled and effectively designed studies on these models will finally bring us to the truth of the iron hypothesis.