Mcs＋血细胞分离机治疗真性红细胞增多症的临床体会

真性红细胞增多症（PV）是一种获得性、克隆性、以红系细胞异常增多为主的慢性骨髓增生性疾病。治疗性红细胞单采术（TE）是采用多功能血细胞分离机去除患者体内增多的红细胞,以达到缓解或减轻临床症状的目的。我科2005年6月-2009年8月运用Mcs＋血细胞分离机进行红细胞单采治疗PV患者14例,短时间内让患者RBC、Hb、Hct接近正常水平,     

治疗性红细胞单采术在真性红细胞增多症患者中的应用

目的:观察治疗性红细胞单采术对真性红细胞增多症(PV)患者的疗效.方法:应用Heamonctics MCS加全自动细胞分离机对8例PV患者行治疗性RBC单采术.8例患者中有7例行2次RBC单采术;1例行3次红细胞单采术.术后复查患者的RBC及红细胞压积(HCT)恢复至正常,同时口服羟基脲治疗,临床症状消失出院.6个月后复查健康状况良好.结果:我们认为对于PV患者应用治疗性红细胞单采术能迅速降低其外周血中的RBC及HCT,术后应用羟基脲利于巩固临床疗效.结论:该方法对PV是一种新的辅助治疗手段,具有临床推广应用价值.     

治疗性血细胞单采术联合药物治疗真性红细胞增多症的疗效分析

目的：探讨血液稀释疗法联合羟基脲等药物治疗真性红细胞真多症的临床疗效及预后评价。方法：对我院收治的真性红细胞增多症患者采用治疗性血细胞单采术去除红细胞2～4次后,立即给予羟基脲等药物治疗。结果：经治疗性血细胞单采术去除红细胞后,患者外周血中红细胞计数明显下降,临床症状好转,再经羟基脲、阿司匹林等药物联合治疗,患者病情得到有效改善。结论：治疗性血细胞单采术联合药物治疗真性红细胞增多症有显著疗效。     

单采红细胞加小剂量化疗治疗真性红细胞增多症

真性红细胞增多症(Polyeythemia.vera,简称真红,PV)治疗方法颇多。我科对3例PV患者应用体外循环单采红细胞,紫外线照射充氧血液回输(ultavio-letirradiation of autotanstustused blood,UVB)配合小剂量化疗方法治疗,取得初步疗效,现报告如下:1 一般资料1.1 病例选择 3例VP患者均为我科住院男性患者,年龄32～72岁。病史资料、血液实验室、骨髓穿刺涂片检查(其中1例为骨髓活检病理检查),符合国内VP诊断标准。     

真性红细胞增多症20例临床分析

真性红细胞增多症（PV）是一种以红细胞增多为主的慢性骨髓增殖性疾病,其发病率低,起病隐匿,常因临床症状不典型而易误诊或漏诊。现就我院2000年1月～2010年5月共收治的20例PV患者进行临床分析,以提高对本病的认识。     

13例真性红细胞增多症临床特征

真性红细胞增多症(PV)属于骨髓增殖性疾病的一种,其发病率较低,起病隐匿,早期症状无特异性,往往未引起人们的注意,患者常在出现较严重的并发症或以其他的症状到医院就诊被发现,有部分患者远期还可能转化成恶性肿瘤,医生起初也往往出现漏诊.     

真性红细胞增多症的诊断及治疗

真性红细胞增多症（polycythemia vera，PV）简称“真红”，是一种获得性，源于造血干细胞的克隆性疾病，其特征为红细胞造血异常增生，红系祖细胞对红细胞生成素高度敏感和非依赖。临床表现有皮肤红紫、脾大、高血压、血栓形成及出血倾向等。血液学特征为红细胞和全血容量绝对增多，血液粘稠度增高，常伴白细胞和血小板增多，晚期可伴有骨髓纤维化或转化为急性白血病。PV、原发性血小板增多症（essential thrombocythemia，ET）、原发性骨髓纤维化（primarymyelofibrosis，PMF）统称为临床上常见的3种BCR／ABL基因阴性的骨髓增殖性疾病（myeloproliferative disorders，MPD）。     

真性红细胞增多症120例临床研究

目的：对多项指标进行分析,提高对本病的认识。方法：真性红细胞增多症是发病率较低的（０．６～１．６／１０ 万）血液病,患者来自我国１７ 省市。着重做了血流变、血动力、微循环及骨髓细胞培养。结果：骨髓生成ＢＦＵ－Ｅ集落数明显高于正常,在无ＥＰＯ情况下仍有较多ＢＦＵ－Ｅ生长。结论：对发病及疗效发挥原理的研究有重要意义。     

真性红细胞增多症并脑梗死1例

<正>真性红细胞增多症(PV)是一种起源于造血干细胞的克隆性、慢性骨髓增殖性疾病,主要表现为以红细胞增多为主的两系或三系血细胞增多。血栓形成是其常见的并发症,多为脑动脉栓塞,常导致腔隙性脑梗死。1病例资料患者,女性,63岁,农民,因双下肢乏力入院。患者于10月30日凌晨起夜发现双下肢乏力,不能行走,伴右侧肢体麻木,无头痛、呕吐、意识障碍,无视物成双、视力下降等不适。既往脑梗死、高血压病史10年余。体格检查:体温:36.7℃,     

Pulmonary hypertension in polycythemia vera

Thromboembolic events occur in about 27% of the patients with polycythemia vera and account for 31% of the deaths. These include cerebrovascular accidents, myocardial infarction, peripheral vascular occlusions, pulmonary infarctions, and venous thrombosis. We report two cases with polycythemia vera who presented with pulmonary hypertension in the absence of previous thromboembolic complications of any kind. One patient died suddenly, with evidence of extensive bilateral thrombosis of prelobular pulmonary arteries at autopsy. In the second patient, local thrombosis in the pulmonary vasculature or recurrent silent pulmonary emboli appear to be responsible for the development of pulmonary hypertension. After institution of anticoagulant therapy, he is able to maintain his functional status. The purpose of this report is to alert clinicians to the development of this insidious, but potentially fatal complication in patients with polycythemia vera. © 1994 Wiley-Liss, Inc.     

Inappropriate erythropoietin secretion in polycythemia vera

A patient with classical polycythemia vera (PV) was found to have an inappropriately elevated serum erythropoietin (Ep) level. Investigations did not reveal any lesion or blood abnormality known to be associated with excessive Ep production and erythrocytosis. Sudden withdrawal of blood to reduce the Hb and Hct from 18.5 gm% and 56% to 13.6 gm% and 41.5%, respectively, resulted in an increment of serum Ep to abnormal level. With iron treatment there was a brisk return of Hb and Hct to prebleeding levels which was associated with reduction in the serum Ep. The inverse relationship between the EP and Hb or Hct is inconsistent with the presence of excessive Ep-producing lesion. These results suggested that the threshold for Ep secretion from normal Ep-secreting tissue to Hb and Hct levels is set at an abnormal level. This patient's marrow cells when cultured in vitro in the absence of Ep, unlike other PV patients' (except one) marrow cells, did not grow erythroid colonies. In the presence of Ep, however, the colonies comparable to those formed from normal marrow cultures were obtained. These results suggested that his marrow erythropoietic cells were neither Ep independent nor Ep-hyperresponsive, as has been suggested by some investigators for erythropoiesis in PV. This patient presents phenomena that hitherto have not been reported.     

单采血小板中红细胞混入量超标原因调查分析

目的:探讨单采血小板中红细胞混入量超标的原因。方法:根据所采集的单采血小板是否红细胞混入量超标(有肉眼可见红细胞),分设正常对照组与超标组,正常对照组为红细胞混入量符合标准的献血者80例;超标组为红细胞混入量超标的献血者23例。分别对2组献血者样本进行全血常规及血红蛋白项目检测。结果:超标组中献血者的Hb、Hct、MCV、MCH及血浆总蛋白结果均低于正常对照组献血者,差异有统计学意义(P0.01);而2组献血者RBC计数、MCHC比较差异无统计学意义(P0.05)。结论:单采血小板红细胞混入量超标与外周血的Hb、Hct、MCV、MCH以及血浆总蛋白等参数的水平降低有关;与献血者多次献血或混合献血造成红细胞未完全成熟有关。     

Endogenous BFU-E in peripheral blood in diagnosis of polycythemia vera

Erythroid progenitors (CFU-E and BFU-E) growth in vitro from bone marrow and peripheral blood of patients with polycythemia vera (PV) was studied using a methylcellulose culture technique. The aim of the study was to find out whether the in vitro colony formation of peripheral blood could be used in the differential diagnosis of PV. In all 25 patients studied, endogenous colonies were found in the bone marrow and peripheral blood. The parallel study of both bone marrow and peripheral blood erythroid progenitors indicates that the presence of endogenous BFU-E in peripheral blood is a dependable test for PV. The results presented here showed that the abnormalities in PV erythroid progenitors are expressed at the level of both CFU-E and BFU-E, suggesting multiple changes in the erythroid progenitors. Our finding indicate that peripheral blood BFU-E differ from bone marrow BFU-E with regard to their dependence for further differentiation on BPA, the activity present in PHA-LCM.     

Increased prevalence of polycythemia vera in parents of patients on polycythemia vera study group protocols

An investigation of relatives of 652 patients entered on studies of the Polycythemia Vera Study Group yielded five documented cases of the disease among the parents of patients. When compared with expected values based on the Connecticut Tumor Registry and other population studies a significant increase was found in the lifetime incidence of polycythemia vera in parents of these patients.     

Incidence of polycythemia vera in a defined population.

Abstract: In this retrospective investigation from Malmö, a city well-suited for epidemiologic studies, 177 patients (88 males and 89 females) with polycythemia vera (PV) were identified between 1950 and 1984. The incidence rate (number of cases/100 000/yr) in both sexes increased significantly, being 1.0 in 1950–1959 and 2.6 in 1980–1984 (adjusted to the European age-standardized population). This is the highest rate reported to date. In 1970–1984 the highest age-specific incidence rates (number of cases/100 000/yr) were found in males ≥ 80 yr and females 70–79 yr of age, being 18.3 and 14.6, respectively. A subgroup of 12 (7%) was identified where the PV diagnosis was not obvious on entry into the study but where it became clear during follow-up. 16 PV patients (9%) had verified or suspected arterial hypoxemia caused by a concomitant condition. We conclude that the increasing PV incidence rates, mainly confined to older age groups, are probably due to better case ascertainment.     

Treatment of polycythemia vera with hydroxyurea

Conventional treatment of polycythemia vera (PV) with radioactive phosphorus or alkylating agents is associated with a significant excess of acute leukemia and cancer of the gastrointestinal tract and skin. There is thus a need for a nonmutagenic agent in the treatment of this disorder. Hydroxyurea (HU) was administered to 118 patients with a loading dose of 30 mg/kg/day for 1 week, which was then reduced to 15 mg/kg/day. Initial control of the elevated hematocrit and platelet count was achieved within 12 weeks in over 80% of patients. Long-term disease control was defined and the accumulative 1-year failure-free survival was 73% in the previously untreated patients and 59% in those patients previously treated with other myelosuppressive modalities. The HU was well tolerated and cytopenia, which generally occurred within the first 8 weeks of therapy, was transient and of little clinical significance. However, it is recommended because of this toxicity that HU be administered initially at a dose of 15-20 mg/kg/day. Three patients developed acute leukemia; two were untreated and one had had myelosuppressive therapy. Hydroxyurea is an effective agent in the treatment of PV, but continued assessment of its mutagenic potential is necessary.     

红细胞增多症与多器官功能损害的临床探讨

目的探讨新生儿红细胞增多症与多器官功能损害的关系及发生率。方法对资料完整的新生儿红细胞增多症96例的临床表现、肝功、胆红索、肾功、心肌酶谱、血糖、心电图、CT检查结果等资料进行分析。结果临床表现以多血貌、黄疸、神经系统及原始反射异常多见。肝功损害以GGT增高较为明显（占85．4％）,GGT增高与胆红素增高有相关性。ALT增高较少（占16．7％）。心肌酶谱异常以AST、CK、CK—MB为主。血糖异常27．1％,低钙血症35．4％,高磷血症69．7％。肾功能检查BUN异常27．1％,Cr异常25．0％。心电图改变以ST-T改变为主;其次为心律改变。CT颅内低密度改变50．2％,出血4．0％。96例中同时有心、脑、肝、肾损害27．8％;同时有2个以上器官损害85．4％。结论新生儿红细胞增多症易引起多器官功能损害,且发生率较高。     

Megakaryoblastic transformation of polycythemia vera associated with hypercalcemia

Leukemic transformation is not uncommon in polycythemia vera, particularly after treatment with chemotherapeutic agents. The leukemias that supervene are mostly of myeloid type but megakaryoblastic transformation is distinctly uncommon. We report a case of polycythemia vera terminating in megakaryoblastic leukemia with associated hypercalcemia.     

Neutrophil marrow egress in polycythemia vera

Summary Neutrophil marrow egress was examined in 6 polycythemia vera patients using³H-thymidine (³H-TDR) pulse labeling. The granulocytopoietic proliferation activity ranged between moderate depression (1420 neutrophils per l blood) and marked enhancement (22470 neutrophils per l blood).Egress characteristics did not significantly deviate from the ones found in subjects with normal granulocytopoietic proliferation activity. Shortest cell sojourn in the medullar nonproliferating granulocytopoietic pool before cell transit into the blood, i.e. neutrophil emergence time, ranged between 67–102 h (median 76 h). After this time interval labeled band and segmented neutrophils simultaneously appeared in the peripheral blood. However, during the early influx phase of labeled neutrophils labeling indices of band forms increased more rapidly than that of segmented forms. In segmented forms the initial curve increment followed approximately an exponential function. Labeling indices doubled in 10–13.5 h with remarkably small variance in the different disease states examined. The results indicate that the main factors influencing neutrophil marrow egress, i.e. maturation of granulocyto-poietic cells, structure of marrow sinusoids and control mechanisms, function normally in polycythemia vera.Supported by grants of the Swiss National Research Fund, No. 3.900.72.