目的:提高对以副肿瘤综合征（PS）为表现的急性髓系白血病（AML）伴嗜碱性粒细胞增多的认识。方法:回顾性分析河北省石家庄平安医院2021年7月收治的1例以PS为表现的AML[慢性粒细胞白血病（CML）转化]伴嗜碱性粒细胞增多患者的临床资料，并复习相关文献。结果:患者，女性，54岁，诊断为CML 42个月，反复出现低血压等PS表现，血常规提示嗜碱性粒细胞增多，骨髓涂片提示AML，第2代酪氨酸激酶抑制剂（TKI）联合化疗未缓解，ABL基因检测到T315I突变，给予第3代TKI联合化疗PS症状改善，嗜碱性粒细胞数量恢复正常，骨髓形态学缓解，最后行异基因造血干细胞移植（allo-HSCT）达到完全缓解，嗜碱性粒细胞比例及数量均正常。结论:以PS为表现的AML患者预后主要与AML预后相关，CML急变为AML发生T315I突变后，一、二代TKI及化疗效果差，预后差，可尝试allo-HSCT改善不良预后。

患者,男,17岁,因"发现皮肤瘀点、瘀斑5个半月"于2019 年8 月30 日入院. 患者自诉 2019 年 3 月 15 日发现皮肤瘀点、瘀斑,外院血常规:WBC计数正常,血红蛋白(Hb)83 g/L, PLT计数31 ×109/L;行骨髓涂片、骨髓活检等(具体结果不详)检查后诊断为骨髓增生异常综合征(MDS)伴多系血细胞发育异常,口服达那唑(每次0. 2 g、每日3 次)治疗5 个月余效果不佳,间断输注PLT,为求进一步治疗转入我院. 既往体健,家族史无特殊.

目的 观察芡莲肠安胶囊联合西药治疗异基因造血干细胞移植(allo-HSCT)后肠道急性移植物抗宿主病(aGVHD)的临床疗效.方法 将54例allo-HSCT后肠道aGVHD患者按照随机数字表法分为观察组与对照组,每组27例.对照组予西药常规治疗,观察组在对照组治疗基础上加用芡莲肠安胶囊,治疗疗程1个月.比较2组治疗前后中医症状评分、肠道菌群变化情况,并评估中医证候疗效和临床疗效,比较2组治疗期间发生肠道等各种感染需要应用抗生素的例数及治疗后见效的时间.结果 治疗后,2组腹痛、恶心呕吐、腹泻频率、食欲减退、四肢无力评分均较本组治疗前显著降低(均P<0.05),且观察组恶心呕吐、腹泻频率、食欲减退、四肢无力评分均较对照组更低(均P<0.05).治疗后,2组肠道菌群失调程度均较本组治疗前好转(P<0.05),且观察组正常(0度)比率高于对照组(P<0.05).治疗后,2组肠道革兰阳性(G+)杆菌菌落计数占比均较本组治疗前显著升高(均P<0.05),G+球菌菌落计数占比均较本组治疗前显著降低(均P<0.05),且观察组G+杆菌菌落计数占比高于对照组(P<0.05),G+球菌菌落计数占比低于对照组(P<0.05).观察组中医证候总有效率为66.67％(18/27),对照组为44.44％(12/27),观察组高于对照组(P<0.05).2组临床疗效、治疗期间发生肠道等各种感染需要应用抗生素的例数及治疗后见效的时间比较差异均无统计学意义(P>0.05).结论 芡莲肠安胶囊联合西药治疗allo-HSCT后肠道aGVHD,能够调节肠道菌群,并明显改善患者的症状.

急性移植物抗宿主病(aGVHD)是异基因造血干细胞移植(allo-HSCT)的主要并发症和死亡原因[1],且肠道是aGVHD攻击最严重的靶器官之一.肠道aGVHD一旦发生,患者极其痛苦,临床症状重,治疗难度大,易合并感染、消化道出血等并发症,甚至危及生命.本文对allo-HSCT后发生消化道出血的8例重度肠道aGVHD患者的临床资料进行分析,以期总结经验,提高肠道aGVHD的诊治能力.

Objective:To investigate the relationship of the acute radiation reactions of totalbody irradiation before hematopoietic stem cell transplantation with the different total and fractionated doses of irradiation.Methods:The clinical data of 48 patients who underwent 6 MV X-ray total body irradiation pretreatment from May 2015 to December 2019 in Shijiazhuang Ping'an Hospital before undergoing hematopoietic stem cell transplantation were retrospectively analyzed. The patients were divided into 8 Gy group (12 cases), 10 Gy group (31 cases) and 12 Gy group (5 cases) according to the total radiation dose, and divided into 4 Gy/f group (17 cases) and 5 Gy/f group (31 cases) according to the fractionated radiation dose. Acute radiation reactions in the oral mucosa, pharynx, salivary glands, upper gastrointestinal tract, lower gastrointestinal tract and lung of patients in each group after radiotherapy were summarized and compared.Results:Acute pharyngeal reaction in the total radiation dose of 8 Gy group showed that 11 cases (91.7%) were grade 0 and 1 case (8.3%) was grade 1; in the total radiation dose of 10 Gy group, 10 cases (32.3%) were grade 0, 13 cases (41.9%) were grade 1, 4 cases (12.9%) were grade 2, 3 cases (9.7%) were grade 3, and 1 case (3.2%) was grade 4; in the total radiation dose of 12 Gy group, 2 cases (40.0%) were grade 0, 1 case (20.0%) was grade 1, 1 case (20.0%) was grade 2, and 1 case (20.0%) was grade 3. The severity of acute pharyngeal radiation reaction in the total radiation dose of 8 Gy group was better than that in the 10 Gy and 12 Gy groups, and the difference was statistically significant ( χ2 = 11.338, P = 0.003); there was no significant difference in the incidence of acute radiation reactions in other parts (all P > 0.05). Acute pharyngeal radiation reaction in the fractionated radiation dose of 4 Gy/f group showed that 13 cases (76.5%) were grade 0, 2 case (11.8%) was grade 1, 1 case (5.9%) was grade 2, and 1 case (5.9%) was grade 3; in the 5 Gy/f group, 10 cases (32.3%) were grade 0, 13 cases (41.9%) were grade 1, 4 cases (12.9%) were grade 2, 3 cases (9.7%) were grade 3, and 1 case (3.2%) was grade 4. The severity of acute pharyngeal radiation reaction in the fractionated radiation dose 4 Gy/f group was better than that in the 5 Gy/f group, and the difference was statistically significant ( Z = -2.606, P = 0.009); there was no significant difference in the incidence of acute radiation reactions in other parts (all P > 0.05). Conclusion:The total dose of 8 Gy and fractionated dose of 4 Gy/f in the total body irradiation before hematopoietic stem cell transplantation can alleviate the acute pharyngeal radiation reaction.

目的 分析芪黄降糖胶囊联合二甲双胍治疗2型糖尿病的疗效及对胰岛素抵抗的影响.方法 选取2016年1月—2017年9月于石家庄平安医院治疗的2型糖尿病患者,共96例,其中48例为对照组,给予二甲双胍治疗,另外48例为观察组,除二甲双胍治疗外,还给予芪黄降糖胶囊.分析2组的治疗效果.结果 治疗后,2组中医症状均得以缓解,且与对照组相比,观察组中医症状评分更低,差异有统计学意义(P<0.05).血糖指标方面,2组的血糖指标显著下降,且与对照组相比,观察组的血糖指标更低,差异有统计学意义(P<0.05).胰岛素相关指标方面,2组的空腹胰岛素(FINS)水平下降,胰岛素抵抗指数(HOMA-IR)得以改善,且与对照组相比,观察组的上述指标更低,差异有统计学意义(P<0.05).不良反应方面,2组比较差异无统计学意义(P>0.05).结论 芪黄降糖胶囊有助于缓解2型糖尿病临床症状、控制血糖,并改善患者胰岛素抵抗状态,用药安全.

目的 探讨移植前乙型肝炎病毒表面抗原(HBsAg)(+)的乙型肝炎病毒(HBV)感染对异基因造血干细胞移植(allo-HSCT)患者粒系和巨核系造血重建、肝功能及HBV免疫标志物的影响.方法 将HBV感染且HBsAg(+)乙型肝炎病毒核心抗体(HBcAb)(+)并行亲缘allo-HSCT的血液病患者10例作为HBsAg(+)组,将年龄、性别、疾病类型和预处理方案等其他情况与HBsAg(+)组相似,但供者和受者均为HBsAg(-)乙型肝炎病毒表面抗体(HBsAb)(-)HBcAb(-)的患者20例作为HBsAg(-)组.收集两组患者造血重建时间及移植前后肝功能并进行比较,观察HBsAg(+)组患者HBV免疫标志物及HBV-DNA变化情况.结果 两组患者在行allo-HSCT后均获得造血重建.HBsAg(+)组与HBsAg(-)组患者粒系重建时间、巨核系重建时间、移植前后肝功能异常率比较差异均无统计学意义(P>0.05).HBsAg(+)组中,6例患者接受HBsAg(-)HBsAb(+)供者的干细胞后,5例实现了HBsAg转阴、HBsAb转阳的血清学转化;移植前有4例受者HBV-DNA(+),移植后除1例HBsAg(+)乙型肝炎病毒e抗原(HBeAg)(+)HBcAb(+)大三阳患者HBV-DNA持续未转阴,其余9例HBV-DNA均为阴性.结论 HBV感染不影响粒系和巨核系造血重建时间,对移植后肝功能影响较小,HBsAg(+)患者接受HBsAb(+)供者的干细胞有利于产生针对HBV的保护性抗体,清除HBV,达到乙型病毒性肝炎临床治愈.

重型再生障碍性贫血(SAA)是一种严重的骨髓造血衰竭性疾病,其病情重、进展快、死亡率高.《再生障碍性贫血诊断与治疗中国专家共识(2017版)》指出,年龄≤35岁且有人类白细胞抗原(HLA)相合同胞供者的SAA患者,如无活动性感染和出血,首选HLA相合同胞供者造血干细胞移植(HSCT)[1-2],其长期生存率可超过80％[3].国外报道找到1例同胞全合供者的概率低于30％,而找到1例合适的匹配无关捐赠者的概率更低,这使同胞全合HSCT开展受到限制[4].近年来由于最佳的治疗方案、改进的支持性治疗和药物的进步,单倍体(HID)造血干细胞移植(SCT)取得显著进展[5-7].本文对近年来SAA的HID SCT治疗现状进行综述.

目的探讨行异基因造血干细胞移植治疗后出现迟发性非感染肺部并发症（LONIPC）特征及诊治。方法选择LONIPC患者4例,均为我院2016年5月至2017年2月收治,分析其临床特征,并回顾诊治效果。结果经2-4周治疗,影像学检查、症状体征均好转,均无病生存。结论迟发性非感染性肺部并发症可能是慢性移植物抗宿主病肺部的一种较为特殊的表现,后者为对前者诊断的重要佐证,在完成移植后,重视肺功能检测和CT检查十分重要,早期应用皮质激素治疗,可有效缓解患者症状。

Resistance to bortezomib (BZ) is the major problem that largely limits its clinical application in multiple myeloma treatment. In the current study, we investigated whether ClC5, a member of the chloride channel family, is involved in this process. The MTT assay showed that BZ treatment decreased cell viability in three multiple myeloma cell lines (ARH77, U266, and SKO-007), with IC 50 values of 2.83, 4.37, and 1.91 nM, respectively. Moreover, BZ increased the conversion of LC3B-I to LC3B-II and expressions of beclin-1 and ATG5, concomitantly with a decreased p62 expression. Pharmacological inhibition of autophagy with 3-MA facilitated cell death in response to BZ treatment. Additionally, BZ increased ClC5 protein expression in ARH77, U266, and SKO-007 cells. Knockdown of ClC5 with small interfering RNA sensitized cells to BZ treatment, and upregulation of ClC5 induced chemoresistance to BZ. Furthermore, ClC5 downregulation promoted BZ-induced LC3B-I to LC3B-II conversion and beclin-1 expression, whereas overexpression of ClC5 showed the opposite results in ARH77 cells. Finally, BZ induced dephosphorylation of AKT and mTOR, which was significantly attenuated by ClC5 inhibition. However, ClC5 upregulation further enhanced AKT and mTOR dephosphorylation induced by BZ. Our study demonstrates that ClC5 induces chemoresistance of multiple myeloma cells to BZ via increasing prosurvival autophagy by inhibiting the AKTmTOR pathway. These data suggest that ClC5 may play a critical role in future multiple myeloma treatment strategies.

目的 研究冠心病患者凝血功能与心血管意外相关性.方法 选取我院2015年2月~2017年2月收治的冠心病患者中的320例为研究对象,对所有患者采用冠状动脉造影进行心血管凝血功能检查,研究凝血功能与心血管意外相关性.结果 随访的结果分为三种,23例死亡;17例为非致死性心血管意外;271例为未发生心血管意外.结论 冠心病患者的凝血功能与心血管意外的发生呈正关系,即凝血功能中的每种指标越高,就越容易发生心血管意外.所以,必须加强对冠心病患者的关注,高度预防高浓度凝血,避免心血管意外出现.

目的:心脑血管疾病中医辨证治疗效果探讨.方法:选取我院2014年5月至2015年5月收治的心脑血管疾病100例,依据数字表抽取法随机分组,就西医常规治疗(对照组,n=50)与中医辨证治疗(观察组,n=50)效果展开对比.结果:观察组冠心病患者28例,临床治疗总有效率经统计为92.9%;脑出血22例,总有效率为95.5%.对照组冠心病患者27例,临床治疗总有效率经统计为74.1%;脑出血23例,总有效率为78.3%,差异均有统计学意义(P<0.05).两组脑出血患者入组时神经功能缺损评分无差异(P>0.05),疗后均有下降,观察组较对照组下降幅度更为显著(P<0.05).结论:心脑血管疾病实施中医辩证治疗可以获得理想的临床效果,具有一定的临床应用价值.

目的：观察原发免疫性血小板减少症（ ITP）患者治疗前后外周血CD4＋CD2＋5调节性T细胞水平变化，探讨凉血解毒方药对ITP患者CD4＋CD2＋5调节性T细胞的作用。方法选取56例ITP患者，男12例，女44例；年龄18～62岁，中位年龄29岁。给以凉血解毒方药治疗（地黄止血胶囊2．0 g，3次／d，口服，凉血解毒中草药煎剂升板汤，1剂／d）。患者于治疗前、治疗后90 d分别采取外周静脉血，采用流式细胞术检测CD4＋CD2＋5调节性T细胞水平。20例健康体检者为正常对照组。结果治疗后第90天，总有效率为73．2％。56例ITP患者治疗前外周血 CD4＋CD2＋5调节性T细胞表达水平低于正常对照组[（1．01±0．67）％，（2．81±0．52）％]，差异有统计学意义（ P ＜0．05）；重症ITP患者CD4＋CD2＋5调节性 T细胞水平低于非重症患者[（0．71±0．23）％，（1．48±0．64）％]，差异有统计学意义（ P ＜0．01）；患者治疗后90 d CD＋4 CD2＋5调节性T细胞水平高于治疗前[（1．93±0．53）％，（10．1±0．67）％]，与治疗前比较差异有统计学意义（ P ＜0．05）；治疗后有效组CD4＋CD2＋5调节性T细胞水平高于无效组，差异有统计学意义（P ＜0．05）。结论 ITP 患者外周血CD 4＋CD 2＋5调节性 T 细胞水平低于正常对照组（ P ＜0．05）；凉血解毒方药提升CD4＋CD2＋5调节性T 细胞水平可能是治疗ITP的疗效机制。

紫癜病崩漏是女性患者由于原发性免疫性血小板减少症（immune thromhocytopenia，ITP，中医称紫癜病［1］）所致的子宫出血的合并症，是血液科常见病症，常因此而造成紫癜病病情加重并导致严重的贫血，甚或因颅内出血或全身广泛大量出血以致急性心力衰竭而死亡。因此，作为紫癜病的严重合并症，探讨紫癜病崩漏有效的快速止血方法有积极的临床现实意义。2009-01-2011-06，我们运用凉血解毒法治疗紫癜病崩漏30例，结果如下。

目的 观察鳖甲生血丸治疗原发骨髓纤维化的临床疗效及不良反应.方法 收集原发性骨髓纤维化患者120例,给予鳖甲生血丸8 g,每日2～3次,口服,共治疗3个月.观察血常规,肝、脾情况,骨髓检查及不良反应. 结果 120例患者好转39例,进步47例,无效34例,总有效率71.67％.30例血红蛋白、白细胞、血小板减低患者治疗后血红蛋白、白细胞显著升高(P＜0.05).白细胞、血小板计数增高的患者,治疗后血小板计数明显降低(P＜0.05).114例脾肿大患者脾大情况治疗后与治疗前比较差异有统计学意义(P＜0.05);81例肝肿大患者肝大情况治疗后与治疗前比较差异无统计学意义(P＞0.05).骨髓病理活检显示治疗后骨髓纤维化程度有明显改善(P＜0.05).120例患者中11例出现少量皮肤出血点,7例有消化道不良反应,减量后消失.结论 鳖甲生血丸治疗原发性骨髓纤维化有较好的近期疗效,无严重不良反应.

In this case head cele was the outstanding clinical manifestation.Head CT images showed slightly high density shadow in the subcutaneous fat of bilateral frontal and temporal departments.The patient initially suffered from thrombocytopenia.Then white blood cells gradually increased and mild anemia occurred.Possible leukemia cell infiltration was found in both bone marrow aspiration and fine needle aspiration of right frontal and temporal cele.Immunophenotyping of peripheral blood cells showed:CD61+43.5%,CD41+36.5%,CD13+37.2% and CD33+41.2%.The patient was diagnosed as acute megakaryocyte leukemia.

Two cases of fellow siblings with myeloproliferative neoplasms were admitted.The sister was diagnosed with overlap syndrome(essential thrombocythemia and myelofibrosis)and the brother was diagnosed with myelofibrosis.Now we will review the incidence,pathogenesis,clinical features and prognosis of familial myeloproliferative neoplasms.

Objective To evaluate the clinical efficacy and safety of allogenic dendritic cells (DCs) and cytokine-induced killer (CIK) cells in the eliminating minimal residual leukemia (MRL).Methods 48 acute leukemia patients with hematological complete remission (CR) but without molecular biological remission (CRM), or patients with minimal residual Leukemia (MRL) were selected from Ping’an Hospital of Shijiazhuang during Jan. 2009 to Jun. 2011. According to the patients’ will, 48 patients divided into combined treatment group and chemotherapy group 24 each. All the patients were in the same general information and disease level. The combined treatment group was treated with DC-CIK and consolidation chemotherapy, and the chemotherapy group was treated with consolidation chemotherapy. PBMCs were collected from healthy donors (the patient's parents or children) to prepare DC-CIK cells. DC-CIK cells were intravenous injected into patients once every 15 days, a total of 4-6 times infusion. The blood routine, bone marrow cells, leukemia related genes, urine and stool routine, liver and kidney biochemistry function, and ECG were observed. Changes of peripheral lymphocyte subsets in patients were detected by flow cytometry. Adverse reactions were examined.Results (1)Eleven cases in the combined treatment group achieved CRM, and the CRM rate was 45.8%; whereas only 2 cases in the chemotherapy group achieved CRM and the CRM rate was 8.3%,the difference was statistically significant(χ2=8.55, P<0.01).(2) Compared with the chemotherapy group, the CFIM (four-color combination flow cytometric immunophenotype of minimal residual leukemia) negative conversion rate of patients in the combined treatment group was significantly raised (66.7% vs 25.0%,χ2=8.39, P <0.01). (3)The negative conversion rate of MRL was higher in the combined treatment group than the chemotherapy group (66.7% vs25.0%, χ2=8.39, P <0.01). (4) After treatment the ratio of CD4+/CD8+ cells was significant increased than before treatment in the combined treatment group (1.3±0.4 vs 0.8±0.4, P <0.05). (5)The complete remission rate (CCR) of patients in the combined treatment group after 3 years was 79.2%, while that in the chemotherapy group was 45.8% (χ2=5.69, P <0.05).(6)No dysfunction of critical organs such as heart, liver and kidney and serious adverse reactions were observed while DC-CIK cells infusion.Conclusion DC-CIK combined with chemotherapy can inhibit leukemia gene, promote the negative conversion rate of CFIM, facilitate the clear of MRL, improve immune function and prolong remission of the patients.No serious adverse reactions were found in patients with DC-CIK infusion.Disclosures: No relevant conflicts of interest to declare.

Objective:To evaluate the clinical efficacy and safety of allogenic dendritic cells(DCs) and cytokine-induced killer(CIK) cells combined with chemotherapy for eliminating minimal residual leukemia(MRL).Methods: Forty-eight acute leukemia patients with morphological complete remission(CR) but molecular complete remission(CRm),or patients with minimal residual leukemia(MRL) were selected from Ping'an Hospital of Shijiazhuang during Jan.2009 to Jun.2011.According to the patients' will,48 patients were divided into combined group and chemotherapy group,with 24 each.All the patients were in the comparable general data and disease level.The combined group was treated with DC-CIK and consolidation chemotherapy,and the chemotherapy group was treated with consolidation chemotherapy.PBMCs were collected from healthy donors(the patient's parents or children) to prepare DC-CIK cells.DC-CIK cells were intravenous injected into patients once every 15 days,a total of 4-6 times infusion.Expression of leukemia specific and related genes were detected by Real-time PCR.Changes of peripheral lymphocyte subsets and MRL immunophentotype in patients were detected by flow cytometry.Adverse reactions were examined.Results: All the patients were followed up to Jun.2012.Compared with the chemotherapy group,the CRm rate of combined group was significantly raised(45.8% vs 8.3% ;χ2=8.55,P<0.01);the four-color combination flow cytometric immunophenotype of minimal residual leukemia(CFIM) negative conversion rate of patients in the combined group was significantly raised(66.7% vs 25.0%,χ2=8.39,P<0.01);the negative conversion rate of MRL was significant higher in the combined group(66.7% vs 25.0%,χ2=8.39,P<0.01);the complete remission rate(CCR) of patients in the combined group after 3 years was significantly raised(79.2% vs 45.8%;χ2=5.69,P<0.05).After treatment the ratio of CD4+/CD8+ lymphocyte was significant increased in the combined group(1.3±0.4 vs 0.8±0.4,P<0.05).No serious adverse reactions were observed after DC-CIK infusion.Conclusion: DC-CIK combined with chemotherapy can inhibit leukemia related gene,promote the negative conversion of CFIM,facilitate the clear of MRL,improve immune function and prolong remission of the patients.No serious adverse reactions are found in patients receiving DC-CIK infusion.