Abnormal hematological indices in cirrhosis

Abnormalities in hematological indices are frequently encountered in cirrhosis. Multiple causes contribute to the occurrence of hematological abnormalities. Recent studies suggest that the presence of hematological cytopenias is associated with a poor prognosis in cirrhosis. The present article reviews the pathogenesis, incidence, prevalence, clinical significance and treatment of abnormal hematological indices in cirrhosis.     

Co hematolog powinien wiedzieć o chorobie Gauchera

Hematological symptoms can be helpful for the diagnosis of inherited metabolic disorders, including Gaucher disease. Gaucher disease is a progressive, multisystem lysosomal storage disorder caused by the deficient activity of the lysosomal enzyme, glucocerebrosidase, arising from autosomal recessive mutations in the GBA1 gene (1q21). Because of constant presence of hematological symptoms in Gaucher disease, hematologists have always been at the forefront of specialists, who performed initial diagnostics of Gaucher disease. Gaucher cells, the lipid-laden storage macrophages, are the pathologic hallmark of Gaucher disease. The clinical presentation of Gaucher disease is highly variable, giving a complex phenotype of multiorgan disease. Classically, three clinical types of Gaucher disease are distinguished according to the absence (type 1) or presence (types 2 and 3) of neurological symptoms and the dynamics of developing clinical signs. Thrombocytopenia, anemia, hepatosplenomegaly and bone manifestations are the most typical signs of type 1, the most prevalent form of Gaucher disease. This paper presents the most important, from the point of view of a hematologist, issues related to symptomatology, diagnosis, and treatment of Gaucher disease.     

重组人血小板生成素治疗化疗相关血小板减少的临床价值

目的 评价国产重组人血小板生成素(rhTPO)治疗实体瘤患者化疗后血小板(PLT)减少的临床疗效和安全性.方法 采用非随机、平行对照的研究方法对化疗后PLT＜75 × 109/L的72例患者进行观察治疗.治疗组35例,采用国产rhTPO治疗,15 000 U/d,皮下注射;对照组37例,采用重组人白细胞介素11(rhIL-11)治疗,3mg/d,皮下注射.结果 用药后,治疗组和对照组PLT的最低值分别为(46.2±20.3)x 109/L和(37.2±16.7)×109/L,PLIT恢复的最高值分别为(250.2±159.0)×109/L和(160.5±96.4)×109/L,两组差异均有统计学意义(均P＜0.05).治疗组Ⅲ、Ⅳ度PLT减少的发生率和持续时间均明显低于对照组(均P＜0.05).治疗组PLT输注4例,对照组PLT输注11例.治疗组不良反应的发生率(11.4%)明显低于对照组(78.4%,P＜0.001),且程度较轻.结论 国产rhTPO能明显地减少PLT降低的程度和持续时间,更快地促进PLT恢复,且患者不良反应轻,安全性好.     

血液肿瘤化疗后血清可溶性白细胞介素-11水平的变化及其意义

目的 观察血液肿瘤化疗后血清可溶性白细胞介素-11(sIL-11)水平与血小板数量改变的关系,探索能维持血小板安全水平的sIL-11临界值,以指导临床治疗.方法 收集血液肿瘤患者化疗前后的血标本,测定sIL-11和血小板水平,观察两者变化间关系并进行统计学分析.结果 99例患者完成研究.化疗后患者的sIL-11水平逐步升高,第6天达到峰值后逐渐下降;血小板数量随时间逐步降低,第10天达到最低值,然后逐渐上升;在血小板达到最低之前sIL-11已达峰值;患者化疗后sIL-11越高,血小板数量越有可能维持于较高水平.根据血小板水半最低值将病例分为两组比较,具较高血小板组有较高sIL-11峰值.其sIL-11平均增长速度快,血小板达到最低值的时间较晚,高sIL-11峰值的病例较多.多元回归显示化疗后血小板低于临界值的影响因素有:sIL-11达到最大值的日平均增长速度和化疗第4天sIL-11低于2000Pg/ml.结论 血液肿瘤患者化疗后sIL-11水平与血小板数量的变化存在相关关系,可以通过测量sIL-11的变化来预测血小板数量的变化趋势.化疗第4天sIL-11<2000 Pg/ml的患者发生严重血小板减少的可能性大,建议给予rhIL-11治疗或输注血小板治疗.     

血小板减少症产妇的护理

目的:探讨血小板减少产妇的护理。方法:对我院住院的3例血小板减少症产妇的临床资料进行分析,总结血小板减少症产妇的治疗和护理体会。结果:无1例发生严重并发症,疗效满意。结论:对血小板减少症的产妇,加强生命体征监测,防止出血及感染,提高母婴生存质量。     

Rapid determination of anti-heparin/platelet factor 4 antibody titers in the diagnosis of heparin-induced thrombocytopenia

PURPOSE: Heparin-induced thrombocytopenia is mediated by antibodies directed against the heparin-platelet factor 4 (heparin/PF4) complex. Our aim was to investigate whether rapid measurement of anti-heparin/PF4 antibodies could improve the diagnostic workup of patients with suspected heparin-induced thrombocytopenia. METHODS: We examined 148 consecutive patients in our laboratory between January 1995 and June 2001 for suspected heparin-induced thrombocytopenia. Clinical data allowed retrospective assessment of the likelihood of heparin-induced thrombocytopenia. Antibodies against the heparin/PF4 complex were detected by a rapid particle gel immunoassay. RESULTS: Anti-heparin/PF4 antibodies were detected in 69 (47%) of the 148 patients, at dilution titers from 1 to 256. Clinically "likely" or "very likely" heparin-induced thrombocytopenia was significantly more common in patients with titers >or=4 (95% [39/41]) than in those with undetectable antibodies (13% [9/70]; P <0.0001), a titer of 1 (18% [4/22]; P <0.0001), or a titer of 2 (33% [2/6]; P = 0.001). All 19 samples with a positive platelet aggregation test had anti-heparin/PF4 antibody titers of at least 4, including 15 samples with titers >or=32. Thromboembolic complications in heparin-treated patients were significantly more prevalent in patients with titers >or=4 (63% [26/41]) than in those with undetectable antibodies (8% [6/79]; P <0.0001) or a titer of 1 (9% [2/22]; P <0.0001). Of the 11 patients with a titer of 1 who were maintained on heparin, none developed worse thrombocytopenia or thromboembolic complications. CONCLUSION: Anti-heparin/PF4 antibody titers, which can be measured rapidly and reproducibly using a particle gel immunoassay, can be used as a confirmatory test to complement a clinical likelihood score among patients with suspected heparin-induced thrombocytopenia.     

Recombinant factor VIIa reduces bleeding in severely thrombocytopenic rabbits

Severe thrombocytopenia is a common complication to intensive chemotherapeutic regimens. For bleeding episodes associated with severe thrombocytopenia, the current standard treatment is platelet transfusion. However, due to several transfusion complications such as transfusion-transmitted diseases, platelet refractoriness and immunomodulation, as well as increasing problems with sufficient supply of platelet products, it is imperative to search for alternatives to platelet transfusion. To test the efficacy of recombinant activated human coagulation factor VII (rFVIIa, NovoSeven) in thrombocytopenia, a preclinical study was conducted in thrombocytopenic rabbits. Thrombocytopenia was induced by a combination of gamma-irradiation and the use of platelet antibodies, and the effect of rFVIIa on nail cuticle bleeding was determined. Administration of rFVIIa at 2 mg/kg significantly shortened the prolonged bleeding time in thrombocytopenic animals (rFVIIa vs. control, median 23 min 41 s vs. 60 min, p=0.016) as well as significantly reducing the blood loss (rFVIIa vs. control, median: 8.8 vs. 12.2 nmol hemoglobin/ml, p=0.016). This effect was also reflected by a significant reduction of the prothrombin time, activated partial thromboplastin time, as well as improvement in clotting parameters in an in vitro thromboelastography thrombocytopenia model. Histopathological evaluation of kidney biopsies for the presence of micro thrombi did not reveal evidence of prothrombotic effects of rFVIIa in this model. These data demonstrate the haemostatic efficacy of rFVIIa in a rabbit model of severe thrombocytopenia. Clinical trials will be needed to further explore the potential of NovoSeven as a haemostatic agent in thrombocytopenic patients.     

A new approach to detect reticulated platelets stained with thiazole orange in thrombocytopenic patients

Recent studies have shown that reticulated platelets stained with Thiazole Orange (T.O.) are useful markers for thrombopoiesis. The percentage of T.O. positive platelets tends to be inconsistent using the original method, especially when the peripheral blood platelet count is very low. We measured T.O. positive platelet levels in patients with severe thrombocytopenic disorders, using concentrated platelet-rich plasma and carrying out a two-color analysis involving T.O. and an anti-glycoprotein IIb/IIIa monoclonal antibody. This method allowed us to obtain consistent T.O. positive platelet rates in patients with thrombocytopenia whose platelet counts were below 20 approximately 30×10⁹/l. By this method, the T.O. positive rates of platelets from idiopathic thrombocytopenic purpura patients were found to be significantly higher than in the control group. The T.O. positive rates of other thrombocytopenic disorders were similar to those of the control group. These results are consistent with those previously reported. We conclude that our technique of measuring of T.O. positive platelets using platelet-rich plasma is useful for analyzing severe thrombocytopenic disorders.     

Ghosal haemato-diaphyseal dysplasia: A new disorder

We describe two siblings, products of a first cousin marriage, with diaphyseal dysplasia, severe anaemia, leukopenia, and thrombocytopenia. Radiologically, both had wide medullary cavities with discrete cortical hyperosthosis. Bone marrow was hypocellular. These, and six similar cases in the literature [6], suggest that they represent a form of diaphyseal dysplasia differing from Camurati-Engelmann disease by their radiological appearance, associated haematological abnormalities and autosomal recessive inheritance.     

旋甘口服液在急性白血病化疗中的应用

目的:观察旋甘口服液在急性白血病病人化疗后升高血小板的效果。方法:选择30例急性白血病患者随机分成两组,均采用标准方案化疗,治疗组口服旋甘口服液30～60ml,1日3次,1个月1疗程。未输注血小板。对照组用黄芪、参麦、益血生,当血小板<20×109/L伴明显出血倾向时输注机采血小板。结果:治疗组血小板上升满意。显效率达80%。全部病例未输注血小板,对照组输注血小板2～5个治疗量/人不等,显效率33%,两组有显著差异P<0.01。结论:旋甘口服液不失为治疗白血病的重要辅助药物。