Hämoptysen

Hemoptysis is a potentially life-threatening complication of various diseases. The most common causes are infectious and inflammatory processes, followed by neoplasms, pulmonary embolisms, mitral stenoses, coagulopathies, and multiple systemic disorders. Primary examinations include chest X-ray, angio CT, and bronchoscopy. Sometimes, a bronchial artery angiogram is required. The patient is at risk of suffocation because blood and clots can severely obstruct his airways. Thus, the most important measures are supplemental oxygen, positioning the patient with the bleeding side down, bronchoscopic suctioning, and removal of blood and clots. Coagulopathies have to be corrected. Application of vasoactive drugs may help temporarily. In cases of bleeding from central lesions, coagulation with laser or argon plasma coagulator is feasible. Heavy bleeding from the periphery requires a balloon or tube tamponade. Depending on the cause and the severity of the bleeding anti-inflammatory medical treatment, hemostyptic radiation therapy, bronchial artery embolization, or a surgical procedure must follow.     

血红蛋白H病

血红蛋白H病或中间型α地中海贫血是以出生后存在大量(5～30％)血红蛋白H(简称HbH)且脾切除患者的许多红细胞中有沉淀的HbH包涵体(本病患者不经脾切除其红细胞内亦多可检得HbH包涵体——编者)为特征的一种溶血性疾病。本文报告502例的研究结果。新生儿患者的临床表现 HbH病的新生儿,无异常表现,肝脾无肿大,血红蛋白水平接近正常,但可见地中海贫血的红细胞形态改变,约25％的患儿可测出HbBait's。HbH仅微量存在,但随后HbH便逐渐代替了Hb Bart's。HbH病的典型表现常在第一年出现。     

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同型半胱氨酸升高是心脑血管疾病的独立预测因素,且与脑卒中关系更密切。伴有血浆同型半胱氨酸浓度升高(>10μmol/L)的高血压被定义为H型高血压,约占我国成年高血压患者的75%。H型高血压的治疗应双管齐下,同时降低血压和血浆同型半胱氨酸浓度。降低同型半胱氨酸最有效的方法是补充叶酸,但同型半胱氨酸降低后是否减少了卒中、冠心病等心脑血管事件仍存在着争议,目前倾向于在一级预防人群中可能有益。携带MTHFR-C677TT基因型的H型高血压患者基线血浆同型半胱氨酸高,可能需强化治疗。     

Hämoptysen

Hemoptysis is a potentially life-threatening complication of various diseases. The most common causes are infectious and inflammatory processes, followed by neoplasms, pulmonary embolisms, mitral stenoses, coagulopathies, and multiple systemic disorders. Primary examinations include a chest x-ray, an angio CT and a bronchoscopy. Sometimes, a bronchial artery angiogram is required. The patient is at risk of suffocation because blood and clots can severely obstruct his airways. Thus, the most important measures are: supplemental oxygen, positioning the patient with the bleeding side down, bronchoscopical suctioning and removal of blood and clots. Coagulopathies have to be corrected. Application of vasoactive drugs may help temporarily. In cases of bleeding from central lesions, coagulation with laser or argon-plasma-coagulator is feasible. Heavy bleeding from the periphery requires a balloon or tube tamponade. Depending on the cause and the severity of the bleeding either anti-inflammatory medical treatment, hemostyptic radiation therapy, bronchial artery embolisation or a surgical procedure must follow.     

Hämatopoetische Wachstumsfaktoren

The production of hematopoietic cells is under the tight control of distinct growth factors. As therapeutic agents, granulocyte colony-stimulating factor (G-CSF), erythropoietin (EPO), and thrombopoiesis-stimulating agents (TSA) are in routine clinical use. Granulocyte colony-stimulating factor is used to prevent febrile neutropenia or to increase dose-density in chemotherapy regimens. Despite a reduced duration of neutropenia, randomized controlled trials have documented only a modest clinical benefit. A clinical advantage of dose-dense chemotherapy has been shown only in specific chemotherapy regimens. Clinical practice guidelines recommend the use of G-CSF for patients with a high risk of adverse outcome of febrile neutropenia. Erythropoiesis-stimulating agents (ESAs) are used as an alternative to blood transfusion in patients with chemotherapy-induced anemia. However, recent meta-analyses of clinical studies suggest that their use was associated with an increased risk of all-cause mortality and serious adverse events. Thrombopoiesis-stimulating agents have been introduced recently into the market for patients with immune thrombocytopenic purpura. Prior to the use of TSA in other conditions such as chemotherapy-induced thrombocytopenia the lessons learned with G-CSF and ESAs should be taken into account.     

H型高血压研究进展

H型高血压是伴有血浆同型半胱氨酸升高的原发性高血压,约占我国成年人高血压的75％,与脑卒中及其他心血管疾病密切相关。降低高血压患者同型半胱氨酸水平对预防卒中有重要意义。     

Hämatoonkologische Krankheitsbilder

Acquired inhibitors of coagulation cause a life-threatening disease. Clinically newly occurring hemorrhagic diathesis combined with prolonged activated partial thromboplastin time (aPTT) time is diagnostically indicative and can be confirmed by a positive plasma exchange test. For thrombotic thrombocytopenic purpura and hemolytic uremic syndrome (TTP-HUS) the diagnosis of Coombs negative hemolytic anemia together with thrombocytopenia should lead to the detection of fragmentocytes in peripheral blood smears. Hairy cell leukemia is a rare subgroup of chronic B-cell neoplasia with the clinical signs of pancytopenia and splenomegaly which characteristically stain positive for CD103. The gastrointestinal stromal tumor (GIST) has nothing in common with classical soft tissue sarcoma based on the activating mutation of the KIT or PDGFRA gene (positivity for CD117). In all of these disorders the correct diagnosis has a major influence on patient outcome. For the case of acquired inhibitors of coagulation immunosuppressive therapy and substitution of coagulation factors (e.g. recombinant factor VIIa) or for TTP-HUS the immediate start of plasma exchange are mandatory. For hairy cell leukemia a very effective treatment exists with purine analogs (e.g. cladribine) and for metastatic inoperable GIST with tyrosine kinase inhibitors (e.g. imatinib).