母细胞性浆细胞样树突细胞肿瘤合并慢性粒-单核细胞白血病1例

母细胞性浆细胞样树突细胞肿瘤是一种来源于浆细胞样树突状细胞前体的侵袭性血液系统恶性肿瘤,临床罕见。本文报道2020年5月福建医科大学附属第二医院收治的1例皮肤母细胞性浆细胞样树突细胞肿瘤合并骨髓慢性粒-单核细胞白血病病例,该患者以皮肤多发结节起病,结合皮肤病理、免疫组化、骨髓细胞学、流式及基因检测获得明确诊断。     

母细胞性浆细胞样树突细胞肿瘤 2例临床病理研究

目的探讨母细胞性浆细胞样树突细胞肿瘤( BPDCN)的临床病理形态特征及诊断与鉴别诊断。方法对泰州市人民医院 2020年住院及 2019年会诊的 2例 BPDCN的临床表现、组织病理学形态及免疫表型进行观察和分析,并复习相关文献。结果 2例病人均为男性,年龄 76岁和 42岁,前者表现为面部单发皮肤结节,后者表现为左颈部淋巴结肿大。镜下,淋巴结内或皮肤真皮及皮下组织见弥漫一致肿瘤细胞浸润,肿瘤细胞中等大小,细胞核卵圆形或不规则,染色质细腻,可见小核仁,胞质稀少,呈母细胞样。 2例病人的肿瘤细胞均表达白细胞分化抗原 4(CD4)、白细胞分化抗原 56(CD56)和白细胞分化抗原 123(CD123),均不表达淋系、髓系及自然杀伤细胞特异性标记,原位杂交检测 EB病毒编码的小 RNA(EBER)阴性。结论 BPDCN是一种少见的高度侵袭性的淋巴造血系统恶性肿瘤,预后差,易累犯皮肤,其诊断需结合临床表现、组织学形态及免疫表型综合分析。     

浆细胞样树突状细胞(pDCs)在慢性乙肝中的研究进展

乙型肝炎病毒在体内持续存在的机制复杂,其中机体细胞免疫功能低下是病毒不能有效清除的重要原因.树突状细胞在清除病毒及肿瘤免疫中发挥激活特异性免疫的效应.浆细胞样树突状细胞(pDCs)是树突状细胞的一个亚型,它是体内产生IFN-α的最主要细胞,并放大对病原体的免疫杀伤效应,从而在抗病毒过程中起重要作用.在慢性乙肝患者外周血中pDCs的功能和数量都有所降低,可能与慢性乙型肝炎病毒不能有效清除有关.本文简述了pDCs基本功能及可能参与慢性乙型肝炎的有关机制.     

急性心肌梗死患者治疗前后外周血树突状细胞亚群变化

目的:探讨急性心肌梗死患者治疗前后外周血树突状细胞亚群数量及比例变化情况。方法:入选病例为我院从2006年1月～2006年12月共20例急性心肌梗死患者;分别留取入院即刻及治疗1周后上述患者外周血标本。借助四色荧光标记技术对外周血髓源性树突状细胞(myeloid DCs,mDCs)和浆细胞样树突状细胞(plasmacytoid dendritic cell,pDC)占DCs细胞比例进行流式细胞术测定。结果:治疗1周后,患者外周血mDCs和pDC占PBMC比例分别为(15.02±7.3)‰、(0.82±0.65)‰,mDC比例较治疗前((4.97±2.7)‰)有显著性差异(P<0.05)。结论:急性心肌梗死患者经治疗1周后,外周血mDC亚群比例和数量明显升高,提示mDC亚群比例和数量的变化与冠心病病情发展密切相关。     

77例浆细胞性乳腺炎分析

目的:探讨浆细胞性乳腺炎的临床表现、诊断、治疗及预后。方法:对1997年7月-2007年10月77例浆细胞性乳腺炎患者临床资料结合文献进行回顾性分析。结果:77例中手术69例,乳管冲洗8例,随访观察时间最长为10年,最短1个月,无复发。结论:浆细胞性乳腺炎患者,临床分型[1]为肿块型,肿块与皮肤粘连,或乳头内陷,临床表现酷似乳腺癌,术前诊断很困难,应尽早手术,常规做病理检查,以免误诊误治。     

浆细胞性龈炎临床诊治探讨(附6例报告)

目的 对浆细胞性龈炎(plasma cell gingivitis)的临床诊治进行探讨.方法 回顾分析6例经治浆细胞性龈炎临床资料.结果 3例经手术切除治疗的局限型患者中2例治愈,1例复发.3例经皮质激素免疫治疗的广泛型患者,症状均明显改善,但其中1例中断治疗后复发.结论 浆细胞性龈炎根据临床表现及病理特点确诊.主要治疗方法有两种:免疫抑制治疗和手术切除.广泛型采用免疫抑制治疗,局限型采用手术切除.     

血管免疫母细胞性淋巴结病1例报告

血管免疫母细胞性淋巴结病(angioimmunoblastic lymphadenopathy,AILD)多发生在老年人,临床具有发热、贫血、多克隆性丙种球蛋白血症及全身肝、脾淋巴结肿大和皮疹。去年10月份我院收治1例,结合本例。将AILD病的发病机理、临床表现诊断、鉴别诊断及目前治疗方法结合文献进行复习。     

浆细胞性乳腺炎23例临床诊疗

目的探讨浆细胞性乳腺炎的临床特点、治疗方法和效果,提高本病的诊疗水平。方法回顾分析我院23例浆细胞性乳腺炎的临床诊疗过程及其治疗体会。结果 23例患者全部行手术治疗,20例治愈,3例术后存在切口红肿、渗液,愈合不佳等情况经保守治疗而愈。结论浆细胞性乳腺炎病情复杂,误诊率高,易反复发作,手术切除是较有效的治疗方法,正确选择手术时机是治疗成功的关键。     

慢性粒细胞白血病并发实体瘤七例报告

既往报道实体瘤后并发白血病较多，而白血病并发实体瘤的报道较为少见，其中慢性粒细胞白血病(CML)初诊时及病程中并发实体瘤的病例更为罕见，近年来我院收治了7例，结合相关献报告如下。     

The advances in therapy of blastic plasmacytoid dendritic cell neoplasm

Introduction: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive myeloid malignancy that contributes to <1% of all hematologic neoplasms. Before the introduction of various targeted agents, the therapeutic approach was based on regimens used for acute lymphoblastic or myeloid leukemia and non-Hodgkin’s lymphoma (e.g. hyperCVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin and dexamethasone alternating with high dose methotrexate and cytarabine) and CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) -based regimens) followed by allogeneic stem cell transplantation for eligible patients. Given that this disease primarily affects older patients, there is a significant barrier to using these highly toxic regimens, even though these regimens are usually associated with the most durable response

Areas covered: In this review, we briefly discuss outcomes with the use of leukemia-based induction regimens as well as the use of stem cell transplant. We also review low-intensity chemotherapeutic regimens. Finally, we will describe both preclinical and early clinical data regarding novel targeted strategies for treating BPDCN without the use of cytotoxic chemotherapy, with a focus on the use of CD123 directed therapy.

Expert opinion: While the current standard treatment for BPDCN is acute leukemia-based regimen followed by hematopoietic stem cell transplantation for transplant-eligible patients, there are very promising results for CD123 directed therapies. The future of BPDCN treatment may include targeted therapies without the need for cytotoxic chemotherapy.     

Tagraxofusp,the first CD123-targeted therapy and first targeted treatment for blastic plasmacytoid dendritic cell neoplasm

ABSTRACT

Introduction: CD123 or interleukin 3 receptor alpha is overexpressed in multiple hematologic malignancies. Tagraxofusp is an intravenously administered CD123-directed cytotoxin consisting of the fusion of interleukin-3 with a truncated diphtheria toxin payload and was recently approved by the Food and Drug Administration for the treatment of adults and children aged 2 and older with blastic plasmacytoid dendritic cell neoplasm (BPDCN).

Areas Covered: In this review, we discuss the use of tagraxofusp in BPDCN, and active clinical trials involving this agent in several hematologic malignancies are also presented. Tagraxofusp has significant efficacy in patients with BPDCN and manageable safety profile, with the most commonly reported adverse events being asymptomatic elevation of alanine and aspartate aminotransferase levels, hypoalbuminemia, peripheral edema, and thrombocytopenia. The most serious side effect is capillary leak syndrome that can be lethal in some cases but the risk may be mitigated by early recognition and intervention.

Expert Opinion: Tagraxofusp has been introduced as a novel treatment of BPDCN, a rare hematologic malignancy, for which no standard therapy previously existed. Many patients treated with this agent were able to be bridged to stem cell transplantation, including older patients. In the future, combinations of tagraxofusp with other targeted agents will be explored.     

淋巴结外指状突树突状细胞肉瘤/肿瘤临床病理观察

目的探讨淋巴结外指状突树突状细胞肉瘤/肿瘤的临床病理学特征及鉴别诊断。方法对1例发生在横结肠系膜的淋巴结外指状突树突状细胞肉瘤/肿瘤进行光镜检查和免疫组化、电镜、流式细胞观察。结果光镜检查,肿瘤细胞由束状、编织状排列的胖梭形、卵圆形细胞和混杂的小淋巴细胞组成,界线不清,胞质略嗜酸性,核呈梭形或卵圆形,核膜清楚,染色质呈细颗粒状,可见核仁。免疫组化:S-100、CD68阳性,SMA、CD20、CD23灶性阳性,CD21、CD45RO、CD34、CD117、EMA、CK阴性,Ki67肿瘤细胞20%阳性。流式细胞:G0/G1(+)、G2(+)、S(+)提示为肿瘤,反应细胞增殖活性较强。电镜:肿瘤细胞具有复杂的指状突起,可见散在溶酶体,无Birbeck颗粒。结论指状突树突状细胞肉瘤/肿瘤是一种罕见的恶性肿瘤,鉴别诊断需借助免疫组化、电镜及流式细胞术。     

树突状细胞介导的CTL在裸鼠体内外抑制白血病细胞生长的研究

目的研究白血病细胞可溶性蛋白抗原（SPA）致敏树突状细胞（DC）介导的细胞毒性T淋巴细胞（CTL）反应在裸鼠体内、外抑制白血病细胞生长的效应,为白血病DC免疫治疗提供理论依据。方法利用人骨髓单个核细胞（MNC）体外培养DC,经形态、表型及功能综合鉴定。用纳米粒包裹白血病细胞SPA并致敏DC,在体外诱导出特异性CTL,用MTT法测定其活性。另分别将HL-60细胞与淋巴细胞（LC）或与CTL同时注入裸鼠体内,通过肿瘤体积、成瘤时间、肿瘤抑制率等对比各组抑制肿瘤生长的效果。结果（1）培养的DC具有DC的形态特征并高表达CD1a、CD83。（2）MTT法测定各组CTL活性,发现A1组较A2组和A3组、B1组较B2组和B3组有明显差异（均P〈0.05）。A3组及B3组CTL活性最高,A2组及B2组活性次之。（3）在裸鼠体内实验中,1组与2组、3组的肿瘤体积及成瘤天数比较均有明显统计学差异（P〈0.05）,而2组与3组比较却无显著性差异（P〉0.05）;2组与3组肿瘤抑制率分别为（50.70±13.74）%和（52.29±13.68）%。结论在体外实验用白血病细胞SPA及纳米粒包裹的SPA致敏DC,均能可介导较强的CTL作用,且纳米粒包裹SPA组的CTL活性更高。用白血病细胞SPA及纳米粒包裹的SPA致敏的DC在裸鼠体内同样可诱导出较强的CTL作用,表现为肿瘤体积明显小于对照组,成瘤天数也明显晚于对照组,但纳米粒包裹SPA致敏DC组在体内试验中未显示出更强的抑瘤活性。通过DC介导特异性的CTL可有效的抑制白血病细胞,是一种有良好临床应用前景的细胞免疫治疗方法。     

雷公藤内酯醇抑制树突状细胞免疫功能

雷公藤制剂在调节机体免疫状态中有重要作用,但仍不清楚雷公藤是否通过ＤＣ而发挥药理作用[1,2]。本实验将研究雷公藤内酯醇对ＤＣ表面人白细胞抗原ＤＲ(ＨＬＡＤＲ,为ＭＨＣⅡ主要成份之一)和免疫分子Ｂ7表达水平及ＤＣ免疫功能的影响,并进一步探讨该影响在雷公藤制剂调节机体免疫状态中的可能作用。1　对象及方法1.1　实验对象及材料　健康成人10例,男女各5例,30～44岁,平均年龄38岁。雷公藤内酯醇由中国医学科学院皮肤病研究所提供。鼠抗人ＨＬＡＤＲ单克隆抗体(ＭｃＡｂ)为ＤＡＫＯ公司产品。鼠抗人ＣＤ83ＭｃＡｂ为ＴｅｄｄｅｒＴＦ博…     

Chemotherapy of acute leukemia in adults

Background: General therapeutic options for adult patients with acute leukemia are reviewed and specific new treatment strategies are described. Objective: Treatment results and controversial issues on current and future antileukemic strategies are discussed. Methods: Data in this review came from the published literature. Results/conclusion: In the past years, striking new developments have been noticeable in the treatment of adult acute leukemia. However, the overall outcome of adult acute leukemia remains poor, particularly in older patients. Intensive chemotherapy remains the standard for leukemia treatment but several approaches using new cytotoxic agents seem promising. Therapeutic targeting of specific biologic abnormalities present in the leukemia cell population might, in a near future, improve outcome of adult leukemia patients.     

1例膀胱血管瘤报道并文献复习

目的提高对膀胱血管瘤的诊治水平。方法回顾性分析I例膀胱血管瘤患者的临床资料并结合文献进行探讨。结果患者行膀胱部分切除术,术后病理检查为膀胱毛细血管瘤,恢复良好,痊愈出院,随访三年未见肿瘤复发。结论膀胱血管瘤是一种罕见的良性肿瘤,彩色多普勒血流显像为膀胱血管瘤首选的无创性检查,膀胱镜检查是诊断本病的主要方法,确诊依赖于病理学检查,治疗方法应视具体情况而定,以膀胱部分切除术为首选。