温抗体型自身免疫性溶血性贫血的诊治进展

自身免疫性溶血性贫血(AIHI)按照自身抗体作用于红细胞时所需的温度分为温抗体型AIHI(WAIHI)和冷抗体型AIHI(CAIHI),以WAIHI居多,并且多为继发性。WAIHI的发病与温度没有显著的相关关系,其发病是由各种原因引起的免疫机制变异,主要包括基因遗传因素、自身免疫调节异常及免疫因素等,导致针对自身红细胞的抗体产生,再与红细胞膜表面抗原结合,它是一种会使自身红细胞破坏或者缩短寿命的比较难以治疗的贫血,目前治疗AIHI首选疗法为肾上腺皮质激素(激素),有效率可达80%,大剂量静脉注射丙种球蛋白(IVIG)、输血、切除脾等方法的使用也在实践中逐渐增多,并且疗效可观。近年来对温抗体型自身免疫性溶血性贫血的诊治进展的研究越来越多。     

The Fanconi anemia/BRCA pathway: a coordinator of cross-link repair

Fanconi anemia (FA) is a rare inherited disease characterized by genomic instability and markedly increased cancer risk. Efforts to elucidate the molecular basis of FA have unearthed a novel DNA damage response pathway, the integrity of which is critical for cellular resistance to DNA cross-linking agents. Despite significant progress in uncovering the molecular events underlying FA, the precise function of this pathway in DNA repair is unknown. This article will review evidence implicating FA proteins in multiple aspects of DNA cross-link repair and propose a model to explain the selectivity of the FA pathway toward DNA cross-linking agents.     

Plasmodium gallinaceum: response of malarious chicken embryos to injection of serum from hyperimmunized chickens.

The injection of serum from chickens hyperimmune to the avian malaria parasite, Plasmodium gallinaceum, or of nonimmune serum into the chorioallantoic cavity of chicken embryos infected with the same parasite resulted in changes in the blood picture of such embryos. In those embryos receiving serum from hyperimmunized birds: The level and rate of parasite increase were depressed; the maturation of the erythroid elements was reduced; the hematocrit values increased only by 27% as compared with 69% in untreated embryos. While hematocrit levels and rates of erythrocytic maturation were depressed in those embryos injected with nonimmune serum, there was no suppression of parasitemia. The results suggest a definite role of immunity in the anemia accompanying malaria.     

罗沙司他治疗肾性贫血的效果观察及对TSAT、Cys C及NOX2的作用分析

摘要 目的：探讨罗沙司他治疗肾性贫血的效果观察及对转铁蛋白饱和度（TSAT）、胱抑素C（Cys C）及NADPH氧化酶2（NOX2）的作用。方法：选择2019年12月到2020年12月在我院接受治疗的125例肾性贫血患者,采用随机数表法分为试验组（n=63）和对照组（n=62）。对照组给予重组人促红素治疗,试验组给予罗沙司他治疗。比较两组临床疗效、TSAT、Cys C、NOX2、红细胞计数（RBC）、血红蛋白（Hb）、血细胞比容（Hct）、铁蛋白（SF）、转铁蛋白（TRF）及铁调素（Hepc）水平变化情况及药物不良反应发生情况。结果：治疗后,两组总有效率比较差异显著（P<0.05）;治疗前,试验组和对照组血清TSAT、Cys C及NOX2比较无显著差异;治疗后,试验组和对照组血清TSAT随着时间的推移而升高,且试验组高于对照组,Cys C及NOX2随着时间的推移而升减降低,且试验组低于对照组,差异显著（P<0.05）;治疗前,试验组和对照组RBC、Hb、Hct检验结果比较无显著差异;治疗后,试验组和对照组RBC、Hb、Hct均随着时间的推移而升高,且试验组高于对照组,差异显著（P<0.05）;治疗前,试验组和对照组SF、TRF及Hepc检验结果比较无显著差异;治疗后,试验组和对照组血清SF、TRF均随着时间的推移而升高,且试验组高于对照组,Hepc随着时间的推移而下降,且试验组低于对照组,差异显著（P<0.05）;两组不良反应总发生率分别为4.76%、8.06%（P>0.05）。结论：在肾性贫血患者中应用罗沙司他效果显著,可能与其可有效改善血清TSAT、Cys C及NOX2水平有关,且不增加不良反应。     

Effectiveness of weekly subcutaneous recombinant human erythropoietin administration for chemotherapy-induced anemia

The effects of weekly subcutaneousrecombinant human erythropoietin (r-hEPO)administration on anemia during chemotherapy includingcisplatin and 5-fluorouracil in patients with head andneck carcinomas were examined. Weekly subcutaneousr-hEPO administration in cancer patients has not beeninvestigated previously. Patients were treated withr-hEPO 100 IU/kg (2 patients), 200 IU/kg (6 patients),or 400 IU/kg (5 patients), or placebo, andeffectiveness was evaluated by monitoring hemoglobinconcentration changes after administration for 8weeks. Hemoglobin concentrations in all 3 r-hEPOdosage groups were higher than that in the controlgroup during chemotherapy. All r-hEPO doses producedimprovements in the anemia induced by chemotherapy;however, the 400 IU/kg dose was most effective. Therequirement for blood transfusions decreased inpatients receiving r-hEPO therapy, and no significantside-effects were associated with r-hEPOadministration. These results suggest thatchemotherapy-induced anemia can be prevented by weeklysubcutaneous r-hEPO administration.     

Microbial Metabolite Signaling Is Required for Systemic Iron Homeostasis

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SLX4: multitasking to maintain genome stability

The SLX4/FANCP tumor suppressor has emerged as a key player in the maintenance of genome stability, making pivotal contributions to the repair of interstrand cross-links, homologous recombination, and in response to replication stress genome-wide as well as at specific loci such as common fragile sites and telomeres. SLX4 does so in part by acting as a scaffold that controls and coordinates the XPF–ERCC1, MUS81–EME1, and SLX1 structure-specific endonucleases in different DNA repair and recombination mechanisms. It also interacts with other important DNA repair and cell cycle control factors including MSH2, PLK1, TRF2, and TOPBP1 as well as with ubiquitin and SUMO. This review aims at providing an up-to-date and comprehensive view on the key functions that SLX4 fulfills to maintain genome stability as well as to highlight and discuss areas of uncertainty and emerging concepts.