Recurrent pneumothorax as a presenting manifestation of active sarcoidosis: a case report and literature review

Sarcoidosis is a systemic granulomatous disease of unknown cause. It commonly affects young to middle-aged adults and frequently presents with bilateral hilar lymphadenopathy, pulmonary infiltration, and ocular and skin lesions. Pleural involvement is relatively uncommon. Pneumothorax is a rare complication of sarcoidosis, occurring usually in the late fibrotic stages of the disease with associated bullous changes. There are only scattered case reports on pneumothorax as the first manifestation of sarcoidosis in medical literature. The present case study reports a young man who presented first with recurrent spontaneous pneumothorax during the early stage and relapse of sarcoidosis.     

An improved anti-leukemic effect achieved with donor progenitor cell infusion for relapse patients after allogeneic bone marrow transplantation

Objective To observe the antileukemic effect in relapse patients by infusion of donor immunocompetent cells with or without granulocyte colony-stimulating factor (G-CSF) mobilization.Methods Twenty patients with leukemia in relapse after allogeneic bone marrow transplantation (allo-BMT) were treated with chemotherapy followed by donor-derived lymphocytes (DDL) without G-CSF mobilization (Group A, n = 11 ), or donor peripheral blood progenitor cells (PBPCs) with G-CSF mobilization (Group B, n =9).Results Five patients in Group A were in hematologic relapse. After DDL infusion, 3 of 5 patients had a temporary complete remission (CR) and relapsed after 3, 7 and 10 months, respectively. One achieved partial remission and died of interstitial pneumonia; and the other one showed no response.Another 6 patients in Group A were in cytogenetic relapse or central nerve system (CNS) leukemia,and all achieved CR and remained in disease free survival (DFS) for 10 to 98 months after DDL infusion. All 9 patients in group B were in hematologic relapse. Three patients with chronic myeloid leukemia (CML) had cytogenetic and molecular remission for 16, 35 and 51 months, respectively after PBPC infusion; and 5 patients with acute lymphoid leukemia (ALL) had CR and were still in CR for 10 to 18 months except 1 patient relapsed soon. And the other one with AML showed no response to the therapy.Conclusion Donor immunocompetent cells infusion is an effective therapy for relapsed leukemia after allo-BMT, especially for the patients with early (molecular and cytogenetic) or CNS relapse. Infusion of donor PBPC mobilized by G-CSF seems to have more potentiated graft-versus-leukemia (GVL)effect than DDL infusion.     

肝细胞性肝癌合并胆管癌栓手术及再手术治疗

Background Few reports have evaluated the efficacy of re-operation for relapse after initial surgery for hepatocellular carcinoma （HCC） with bile duct thrombosis （BDT）.The aim of this study was to investigate the efficacy of initial surgery and subsequent re-operation for HCC with BDT, and their effects on prognosis.Methods The clinical data of 880 patients with HCC, including 28 patients with BDT, who underwent radical hepatectomy between 1998 and 2008 in our hospital, were reviewed.The effects of BDT and re-operation on prognosis were retrospectively analyzed.Results The 1-, 3- and 5-year survival rates were 89.3%, 46.4% and 21.4%, respectively, in 28 patients with BDT versus 91.4%, 52.9% and 20.9% in 852 patients without BDT （P 〉0.05）.Six patients with BDT underwent re-operation after disease relapse, and their survival time was significantly longer than those who did not undergo re-operation （P 〈0.05）.Multivariate analysis indicated that portal vein invasion and tumor size were independently associated with tumor relapse and prognosis （P〈0.05）.Univariate analysis and multivariate analyses showed that obstructive jaundice was not significantly correlated with tumor relapse or prognosis （P 〉0.05）.Conclusions Hepatectomy plus BDT removal is an effective treatment option for HCC with BDT.Obstructive jaundice is not a contraindication for surgery.Re-operation after relapse can provide good outcomes if the cases are appropriately selected.     

Study of cultured bovine capsular bag in pure ocular tissue

The proliferation, differentiation and fibrosis of lens epithelia cells （LECs） is mainly responsible for posterior capsular opacification （PCO）. From the primary culture of LECs to the culture of lens capsular bag, the models of posterior capsular opacification have been developed. At present, the most commonly used model is cell culture in medium with serum. But the culture in pure ocular tissue has not been reported. Therefore, we established a new model of posterior capsular opacification-culturing bovine lens capsular bag in pure ocular tissue to exclude the role of serum. Our study established a new culture method to investigate the proliferation, differentiation and apoptosis of lens epithelia cells in the aqueous humor with or without lens cortex and vitreous humor. The purpose of the study is to model posterior capsular opacification in vivo as closely as possible and to discuss the influence of ocular tissue on posterior capsular opacification.     

The evaluation of Tracp5b as a marker for monitoring treatment results of bone metastasis in breast cancer patients

Objective ：To evaluate the sensitivity of serum tartrate-resistant acid phosphatase 5b（Tracp5b） activity in monitoring bisphosphonate treatment results of bone metastasis in breast cancer（BC） patients. Methods：The serum activities of Tracp5b, CEA, CA153 were measured in 58 BC patients, including 26 without bone metastasis, 32 with bone metastasis. The serum activities of TracpSb, CEA, CA153 were also measured in 19 patients with bone metastasis after 3 months of bisphosphonate treatment. Eighteen healthy women with age from 34 to 70 served as control. Results：Serum TracpSb was significantly elevated in patients with bone metastasis compared with that in all any other groups（P〈 0.05）. The sensitivity of TracpSb was 78.13% and the specificity was 86.36%. The sensitivity of CA153 was 37.50% and the specificity was 77.27%. The sensitivity of CEA was 21.88% and the specificity was 84.09%. The serum activity of TracpSb decreased significantly（P 〈 0.05） after 3 months of bisphosphonate treatment, while the levels of CA153 and CEA were unchanged. Conclusion：Serum Tracp5b activity is a useful diagnostic marker for bone metastasis in BC patients and can be used to evaluate the treatment results of bisphosphonate.     

A comparative study of calcium sulfate artificial bone graft versus allograft in the reconstruction of bone defect after tumor curettage

Background Cavity reconstruction after benign bone tumor removal is varied and controversial.AIIograft is widely used but is associated with complications.New bone substitutes,such as calcium sulfate artificial bone,have been introduced for bone tumor operation.However,the bone healing response of artificial bone has not been compared with allograft bone.We therefore compared calcium sulfate grafts （study group） with bone allografts （control group） for the treatment of benign bone tumors.Methods We retrospectively reviewed 50 patients who underwent calcium sulfate reconstruction and 50 patients who underwent allograft cancellous bone reconstruction.The two groups were well matched.The mean follow-up time of the study group was 19.9 （12-55） months.We investigated bone healing response,complications,and factors affecting bone healing.Results At the last follow-up,84％ （42/50） of cases in the study group and 62％ （31/50） of cases in the control group had achieved clinical healing （P=0.013）.The initial healing rate showed no significant difference between the two groups （100％ vs.96％,P=0.153）.The mean healing times for calcium sulfate and allograft bone were 9.6 （3-42） months and 13.8 （3-36） months,respectively （P ＜0.01）.Complications in the study group were minor and resolved.Implant volume was a significant factor affecting bone healing.Conclusion The calcium sulfate bone substitute showed a satisfactory healing outcome and safety profile in reconstruction of bone defects after benign bone tumor curettage,especially in smaller cavities.     

Repair of Rabbit Femoral Defects with a Novel BMP2-derived Oligopeptide P24

In this study, the bioactivity of a novel BMP2-derived oligopeptide P24 was investigated by using the model of rabbit femoral defect after loaded in the biodegradable poly （lactic acid / glycolic acid / asparagic acid-co-polyethylene glycol） （PLGA-[ASP-PEG]）. A 1.5-cm unilateral segmental bone defect was created in the left femoral diaphysis in each of the 30 new zealand white rabbits. The defects of 18 legs filled with BMP2-derived peptide P24 combined with PLGA-[ASP-PEG] scaffold serves as the experimental group, and the defects in the rest 12 rabbits filled with （PLGA-[ASP-PEG]） without P24 as control group. The bone-repairing capability in the target region of the two group was grossly, radiologically, histopathologically and biomechanically evaluated 4, 8 and 12 weeks after the operation. Our results showed that in each group, primary healing of incision was achieved in the two groups. Radiographically, in experimental group, defects were filled with induced callus within 8 weeks, and a cortical bone-like structure was observed in some animals at the 12th week. According to the standardized stage of bone defect repair, 9 （64.28%） achieved grade-4 healing. In contrast, little bone formation was seen in the defects even 12 weeks after the operation, and 5 （62.50%） had grade 0 healing in this group. Histologically, tissue engineering material was mostly absorbed and cartilage was found around implants in the experimental group at the 4th week; 8 weeks after operation, the engineering material was completely absorbed, and formation of woven bone was observed and typical trabecular bone structure could be seen. In control group, 8 weeks after operation, the defect was filled with fibrous tissues, and no bone-like structure was observed. Statistical analysis showed very significant difference in biomechanical indicators between the two groups （P〈0.05）. It is concluded that new oligopeptide P24 can induce excellent bone regeneration and promote bone repair.     

Asymptomatic Paget’s disease of bone presenting with complete atrioventricular block

Paget＇s disease of bone is a deforming bone disease （osteitis deformans） characterized by increased bone remodeling, bone hypertrophy, and abnormal bone structure, leading to bone expansion, deformities, easy fractures, and occasionally, neoplastic transformation. It is the second most common bone disorder after osteoporosis. The disease is relatively rare in Asia but is common in Europe and North America, affecting approximately 2% of the population over 50 years,     

Interferon α: the salvage therapy for patients with unsatisfactory response to minimal residual disease-directed modified donor lymphocyte infusion

Background Minimal residual disease （MRD）-directedmodified donor lymphocyte infusion （mDLI） is used to treat relapse after hematopoietic stem cell transplantation （HSCT）. For patients who experience an unsatisfactory response tomDLI relapse is usually inevitable. Therefore we sought to evaluate the efficacy ofinterferon a therapy in these patients. Methods Regular MRD monitoring was carried out after the HSCT. The patients who were MRD-positive underwent mDLI. Patients with an unsatisfactory response to mDLI received interferon a therapy （3 million units, twice weekly） with regular monitoring of MRD. To ensure the immunomodulatory effects of interferon a, immunosuppressant treatment would be stopped before interferon a treatment. Results Five patients with an unsatisfactory response to mDLI treatment received interferon a （3 had t（8;21） chromosomal translocation acute myeloid leukemia, and 2 had common acute leukemia）. They had significantly reduced or resolved MRD. Four patients developed chronic graft-versus-host disease. Two of the 5 patients reported transient fevers, and no significant bone marrow suppression was observed. All of them were in continuous complete remission after interferon a treatment. The median survival time was 469 days （range 368-948 days）. Conclusions In patients with an unsatisfactory response to MRD-directed mDLI, interferon a may directly or indirectly induce the graft-versus-leukemia effect to improve mDLI efficacy and clear MRD. Chin Med J 2014;127 （14）： 2583-2587     

Metastatic tumor to the iris and ciliary body as an initial sign of lung cancer: a case report

The most common sites of lung cancer metastases are pleura, bone, brain,pericardium and liver. Tumor metastasis to the eye is a rare complication of lung cancer. Metastatic cancer to the ocular region most often involves the uveal tract, however, most uveal metastases occur in the posterior uvea, and iris metastases are relatively rare. We describe a patient with adenocarcinoma of the lung metastatic to the iris as the first clinical sign without symptoms of lung cancer.     

FLT3基因突变与急性早幼粒细胞白血病髓外复发

目的 探讨 [QX(Y9]FLT3[QX)]基因突变在急性早幼粒细胞白血病（acute promyelocyticleukemia,APL）髓外复发中的意义。 方法 回顾性检测2例典型APL髓外复发患者骨髓及血液标本可能存在的基因突变,分析FLT3突变与APL髓外复发的相关性。结果 1例APL中枢神经系统复发的患者发生较罕见的FLT3-酪氨酸激酶结构域（TKD）点突变Asn841Gly（A841G）及一种新的WT1基因突变 (c.1209_1210insT/p.K404X);1例APL睾丸复发的患者发生较罕见的FLT3-TKD点突变Asp839Gly (D839G)和一种新的WT1基因突变Arg458Pro (c.1373G>C)。 结论 本研究病例中发现的罕见FLT3突变与新的WT1基因突变可能与APL髓外复发相关,为后续相关研究提供了基础。临床中需重视APL患者基因突变检测,以便更好分层治疗,改善预后。     

异基因外周血干细胞移植治疗高危白血病

目的　探讨异基因外周血干细胞移植(allo-PBSCT)治疗高危白血病的疗效。方法　25例高危白血病患者,中位年龄34（5.5～52）岁,接受了HLA配型相合同胞供者外周血干细胞移植,其中急性白血病（AL）15例,（第一次完全缓解1例,为Ph染色体阳性,第二次及以上完全缓解期7例,复发7例,包括2例异基因骨髓移植(BMT)后复发;慢性粒细胞性白血病（CML）4例;（第2次慢性期、加速期、急变期、BMT后复发各1例）;骨髓增生异常综合征(MDS)6例,移植物抗宿主病（GVHD）预防方案采用经典环孢霉素（CsA）加氨甲蝶呤（MTX）。结果　所有患者均植活,中性粒细胞数恢复至≥0.5×109/L和血小板数≥20×109/L的中位时间分别为移植后14(10～18) d和11（7～45） d。发生II度及以上急性GVHD13例,包括1例III度GVHD,未发生IV度急性GVHD。23例可评估患者中16例(70%)诊断慢性GVHD。移植相关死亡率为16%,复发6例,4例经回输供者淋巴细胞获得再次缓解。19例患者无病存活,中位随访时间为304（94～1 963） d。2年总生存率、无病生存率及复发率分别为64%、58%及25%。结论　allo-PBSCT可治疗高危白血病患者,降低移植后复发率,延长无病生存。对有高危因素的血液系统恶性肿瘤患者,选择PBSCT替代骨髓移植更有优势。     

Paranasal sinus involvement in acute lymphoblastic leukemia

Acute lymphoblastic leukemia (ALL) is a hemopoietic malignancy of the bone marrow that rarely invades the sinonasal area. If infiltration of paranasal sinuses occurs, it may lead to rhinosinusitis and orbital complications that need aggressive treatment. In this report, a 26-year-old male patient who had a history of ALL and had one relapse, suffered from rapid progression of right periorbital pain and exophthalmos, which usually presented in patients with orbital complications of sinusitis. A sinus computed tomography showed right maxillary and ethmoid sinus opacification with orbital bone destruction. Urgent endoscopic sinus surgery (ESS) was performed for orbital decompression and histopathologic diagnosis. Pathology revealed lymphoblast infiltration and inflammation of respiratory mucosa. Subsequent bone marrow aspiration cytology confirmed the diagnosis of a second relapse of ALL. Obstructive causes of sinusitis should be evaluated in patients with ALL. ESS has proven to be effective in obtaining tissue for histopathologic diagnosis, and in treating patients with sinusitis with orbital complications.     

儿童急性淋巴细胞性白血病细胞凋亡与临床相关性研究

①目的　探讨急性淋巴细胞性白血病病儿细胞凋亡与临床分型及预后的关系。②方法　采用AO EB荧光染料双染法 ,检测 30例初治急性淋巴细胞性白血病 (ALL)病儿治疗前后不同时期骨髓单个核细胞凋亡指数 (AI)。③结果　ALL病儿治疗前AI明显低于正常对照组 (t=5 .4 17,P <0 .0 1) ,标危型治疗前AI高于高危型(t=5 .0 0 2 ,P <0 .0 1)。治疗前AI与外周血WBC呈负相关 (r =- 0 .6 32 ,P <0 .0 1) ;泼尼松及诱导缓解治疗后 ,随着细胞凋亡水平的升高 ,WBC逐渐下降 ,标危型组WBC下降明显 (t=3.14 8,P <0 .0 1) ;标危型病儿经诱导缓解期治疗完全缓解率高 (P =0 .0 0 2 5 ) ;随机动态观察标危型及高危型ALL病儿各 5例 ,高危型病儿 3例出现中枢神经系统白血病 ,2例骨髓复发 ,而 5例标危型骨髓检查为持续完全缓解 ,并且无髓外浸润表现。④结论　细胞凋亡可作为评价化疗反应及预后的参考指标之一。     

成人急性淋巴细胞白血病的治疗现状及展望

近年来随着科学的发展,成人急性淋巴细胞白血病的治疗有了很大的进展。诱导治疗至少应用一次糖皮质激素+长春新碱+蒽环类药,尽量加用门冬酰胺酶,强烈的诱导治疗必须权衡利弊。在强烈的诱导和巩固后维持治疗仍然是急性淋巴细胞白血病患者的标准治疗。高危患者在第一次完全缓解时推荐干细胞移植,标危者不推荐。中枢神经系统白血病的预防性治疗是防止中枢神经系统受累或复发的基础。总之,成人急淋应根据预后因素及患者特点等确定个体化治疗。     

小儿急性白血病CD34抗原表达的临床意义

目的探讨儿童急性白血病CD34抗原表达的临床意义。方法采用流式细胞术对78例急性淋巴细胞白血病(ALL)及27例急性髓系白血病(AML)进行免疫表型分析。结果 78例ALL患儿CD34阳性率为43.6%,27例AML患儿CD34阳性率为29.6%,CD34+组与CD34-组在性别和初诊时白细胞计数比较差异无统计学意义(P>0.05);CD34+组与CD34-组在肝脾淋巴结肿大等髓外浸润表现、诱导治疗4周的缓解率上差异有统计学意义。结论 CD34抗原表达在临床特征上主要表现在髓外浸润,为化疗反应差的影响因素。     

以口腔粘膜细胞为对照检测异基因造血干细胞移植后嵌合状态

目的：探讨以口腔粘膜细胞DNA代表移植前基因状态的可行性，解决缺乏移植前样本病例嵌合状态的检测问题．方法：对2例接受异基因造血干细胞移植术病例，采集供者、受者移植术前外周血、受者移植术后外周血和口腔粘膜拭子，以PCR-STR技术对各样本进行DNA分析，对比移植前后基因分型变化判断嵌合状态，比较受者移植术前外周血和移植术后口腔粘膜拭子基因分型的异同．结果：2病例移植后受者外周血的PCR-STR基因分型与供者一致，呈完全的供者嵌合状态；受者移植后的口腔粘膜细胞基因分型与移植前外周血的基因分型一致．结论：口腔粘膜细胞的DNA样本可以代表受者移植前的基因状态，在实践中可以用口腔粘膜细胞代替移植前的受者样本进行嵌合状态的检测。     

急性淋巴细胞白血病移植瘤模型小鼠髓外浸润过程中血清Wnt5A和LINE-1启动子甲基化水平检测及其意义

目的:建立绿色荧光蛋白(GFP)标记的急性淋巴细胞白血病(ALL)移植瘤小鼠模型,研究ALL髓外浸润过程中血清游离DNA(cfDNA)特异基因甲基化的变化,为ALL髓外浸润监测提供理论和实验依据.方法:60只ICR小鼠随机分为对照组、实验15 d组和实验30 d组,每组20只.实验组小鼠通过尾静脉注射构建GFP标记AL...