CD34-Negative B-Lymphoblastic Leukemia/Lymphoma Presenting as Cutenous Lesions at Infancy: A Case Report

Acute lymphoblastic leukemia/lymphoma is a highly aggressive neoplasm of precursor lymphoid (blast) cells. There are 2 main subtypes based on lymphoid lineage;B lymphoblastic leukemia/lymphoma (B-ALL/LBL) and T lymphoblastic leukemia/lymphoma (T-ALL/LBL). B-ALL/LBL commonly presents with fever, fatigue, bone or joint pain, bleeding or anorexia (signs of bone marrow infiltration), lymphadenopathy, hepatosplenomegaly, involvement of skin, soft tissue and testes, with a predilection for the central nervous system. Immature cell markers, such as CD34 and TdT, can help to differentiate lymphoblasts from Burkitt lymphoma which, is considered a mature high-grade B cell lymphoma that mimics lymphoblastic lymphoma/leukemia. Unfavorable prognostic factors include: infancy and adult age of diagnosis, high white blood cell count, slow response to initial therapy, central nervous system involvement at the time of diagnosis and Minimal residual disease after therapy. We present a case report of a 4 months old infant seen at a Tertiary Hospital with a rare presentation of CD34 Negative B-lymphoblastic leukemia/lymphoma presenting as cutaneous lesions in infancy.

Primary cutaneous anaplastic large cell lymphoma with subsequent leg involvement

Primary cutaneous anaplastic large cell lymphoma(CALCL)is regarded as an indolent type of cutaneous T-cell lymphoma.However,a few recent publications revealed that C-ALCL patients with initial leg involvement had significantly worse survival than those without initial leg involvement.Herein,we report a case of C-ALCL with subsequent leg involvement,which led to death after chemoradiation therapy.A 75 years old Japanese man presented with multiple erythematous nodules in his left arm and the side of his left chest.Histopathological and immunohistochemical studies led to the diagnosis of primary C-ALCL.At the initial diagnosis,no leg lesion was found.One year after the initial diagnosis,C-ALCL appeared in his right lower thigh and left hip.Radiation therapy,low-dose etoposide and CHOP therapy were performed;however,the patient died of malignant lymphoma 4 years after the initial diagnosis.We speculated that the occurrence of subsequent leg involvement may also be indicative of a worse prognosis,as in the case with initial leg involvement in C-ALCL.Therefore,we propose that C-ALCL patients with initial or subsequentleg involvement should be classified as a distinct clinicopathological variant of C-ALCL("leg-type"involvement)and that they may require intense therapy.

Expressions of Antimicrobial Peptides LL-37, Human Beta Defensin-2 and -3 in the Lesions of Cutaneous Tuberculosis and Tuberculids

Background： Antimicrobial peptides, including cathelicidin LL-37, human beta defensin （HBD）-2, and HBD-3, are important elements of the innate immune response and involved in modulation of the adaptive immunity, and they also play an important role in cutaneous defense against Mycobacterium tuberculosis. Methods： The fresh skin tissues and paraffin-embedded biopsy samples from three cutaneous tuberculosis, two tuberculids, and ten healthy individuals were collected. The expressions ofLL-37, HBD-2, and HBD-3 mRNA in the lesions of three cutaneous tuberculosis and two tuberculids were detected by quantitative real-time polymerase chain reaction; the protein expressions were detected by immunohistochemistry and Western blotting methods. Results： The expressions of LL-37 mRNA and protein in the lesions of cutaneous tuberculosis and tuberculids were similar to that of normal skin. The expression of HBD-2 mRNA had an increasing trend in the lesions of cutaneous tuberculosis and tuberculids compared with that of normal skin; however, the expression of HBD-2 protein in the lesions of cutaneous tuberculosis had a decreasing trend compared with that of normal skin, and the expression of HBD-2 protein in the lesions of tubereulids was similar to that of normal skin. The expressions of HBD-3 mRNA and protein in lesions of cutaneous tuberculosis and tuberculids were similar to that of normal skin. Conclusions： Our study indicated that the expression of HBD-2 and HBD-3 mRNA and protein in lesions of cutaneous tuberculosis may be not consistent with that of tuberculids. However, an inherent limitation of the present study was that the sample size was small, and the roles and regulation mechanisms ofLL-37, HBD-2, and HBD-3 in cutaneous tuberculosis and tuberculids need to be further investigated.

Cutaneous lesions and disorders in healthy neonates and their relationships with maternal-neonatal factors: a cross-sectional study

Background:Cutaneous lesions are very common in neonates.Although a number of studies have reported on their incidence,very little is known about the factors that influence them.We set out to investigate a large population of neonates with the aims of achieving an overall picture of neonatal skin manifestations,and examining their relationships with various maternal,neonatal and perinatal factors.Methods:This study was conducted on neonates born at the Department of Obstetrics and Gynaecology at the University of Szeged between June 2013 and July 2015.A total of 4658 consecutive infants underwent a whole-body skin examination within the first 72 hours of extrauterine life.The official neonatal medical charts were used to collect data on the history of the participating neonates and on maternal factors.Results:74.35% of the neonates exhibited at least one skin manifestation.The major diagnosis groups were transient,benign cutaneous lesions;vascular lesions;traumatic,iatrogenic,congenital or acquired disorders with skin injuries;pigmented lesions;and developmental abnormalities or benign skin tumours.The relationships between the skin findings and six neonatal or maternal factors were examined:gender,gestational age and birth weight of the neonates;maternal age and the number of previous pregnancies of the mothers,and mode and circumstances of the delivery.Conclusions:We found several significant correlations between the examined maternal/neonatal factors and the occurrence of birthmarks and neonatal skin disorders.Of course,further studies are required to confirm and better understand these associations.

Behçet Disease with Generalized Polymorphous Cutaneous Lesions:A Case Report

Introduction:Behçet disease is a chronic multisystem vasculitis disease,however generalized polymorphous cutaneous lesions are uncommon.Here,we reported a case of Behçet disease with multiple parts of the body and complex lesions,which may lead to misjudging in clinical diagnosis.Case report:A 69-year-old man presented with a seven-year history of recurrent generalized polymorphous mucocutaneous lesions(erythematous papules,nodules,ulcers,and necrosis)over his entire body,and the lesions had been painful for the past three years.Based on the past medical history,clinical presentation,histological examination excluded other diseases,and the 2014-amended International Criteria for Behget disease,the patient had a score of 6 points and was diagnosed as Behçet disease.Discussion:The common clinical feature in patients with Behçet syndrome is the presence of recurrent and usually painful mucocutaneous ulcers.Other clinical manifestations of this disorder are more variable among different patients.A diagnosis of generalized polymorphous cutaneous lesions should remain on the list of differential diagnosis of Behçet disease after excluding other diseases.Conclusion:The mucocutaneous lesions of Behçet disease are often preceded by other manifestations,and timely diagnosis may benefit early treatment and prognosis.

The Quest for Understanding Cutaneous Leishmaniasis in Northern Province,Sri Lanka:An Analysis of Clinical Data From the District General Hospital,Vavuniya

The study aimed to examine the clinical and epidemiological patterns of cutaneous leishmaniasis(CL)in patients attending the Dermatology Unit,District General Hospital in Vavuniya,Sri Lanka.A total of 77 patients clinically suspected for CL were interviewed through a structured questionnaire,and skin-lesion samples were obtained between January 2016 and January 2017.The definitive diagnosis of CL was made through microscopic identification of smears,histopathological examination of biopsies and kDNA PCR.Treatment modalities were chosen based on the location of the lesions on the body and complexity of the lesions.Of 77 suspected patients,68 were confirmed for CL,with a mean age of 34.6(±12.7)years,and included 54 males(79.4%)and 14 females(20.6%).Being a male was a significant risk factor(P=0.032,OR=4.82)associated with CL.Lesions were observed mainly on the exposed areas of the body,of which the forearm(22.1%)was the most commonly affected site.Single lesions(75.0%)and ulcerated nodules with central crust(39.7%)were the prominent features among the infected group.The age group of 21–40 years was significantly associated with healing lesions(P=0.028,marginal effect=0.55).However,a significant negative relationship was detected between ulcerated nodular lesions and lesion healing(P=0.0436,marginal effect=−0.375).Males are at higher risk of CL.Early diagnosis and specific treatment,along with preventive measures such as protective clothing and sand fly repellents,can limit the spread of the disease.There is a need for a comprehensive approach to prevent and control the transmission of CL.

皮肤Rosai-Dorfman病的皮损临床表现及组织病理特征14例分析

目的:探讨皮肤Rosai-Dorfman病(CRDD)的皮损临床表现特征,结合组织病理、免疫表型和鉴别诊断可提高CRDD的正确诊断。方法:收集14例CRDD患者资料,分析其临床表现特征并进行归类分型,对标本进行组织病理和免疫组化染色观察。结果:14例CRDD患者的皮损表现可分为丘疹结节型(64.7%),浸润斑块型(5.9%)及肿瘤样型(29.4%)3种基本类型,无淋巴结增大等其他系统性病变。组织病理和免疫表型特点为真皮和皮下脂肪组织内数量不一的S-100蛋白阳性大型组织细胞,伴大量以淋巴细胞和浆细胞为主的混合性炎性细胞浸润,组织细胞胞质内常吞有数量多少不一的淋巴细胞。1例脉管内可见组织细胞。少数患者出现淋巴滤泡、纤维化及脂膜炎样和血管炎样组织病理形态。结论:CRDD皮损表现多种多样,3种基本皮损类型的分型方法能提高临床对CRDD诊断的皮损鉴别能力,结合组织病理改变和免疫表型可与多种皮肤病和皮肤肿瘤作鉴别。CRDD可复发,应作长期随访。

皮肤镜在诊断色素性疾病方面的研究

目的研究皮肤镜在诊断色素性疾病方面的应用。方法回顾性分析2002年1月至2006年12月470例经病理证实的色素性疾病的皮肤镜所见。结果在470例皮损中有108例Clark痣,179例脂溢性角化病,128例基底细胞癌,50例恶性黑素瘤,5例蓝痣。其中,利用皮肤镜的模式分析法93.6%的脂溢性角化病得到正确诊断,93.3%的基底细胞癌、83.5%的恶性黑素瘤、91.2%的蓝痣得到正确诊断。结论皮肤镜为不同色素性疾病的鉴别提供诊断依据,对不同色素性皮损诊断的敏感性和特异性很高,可以提高色素性疾病的正确诊断率。

带状疱疹急性期临床症状与皮损区病理性改变的相关性研究

目的观察带状疱疹急性期临床症状与皮损区病理性改变的相关关系,以及皮损区早期应用抗免疫炎性治疗对病程转归的影响。方法①基础研究部分:选取临床确诊的带状疱疹病人3例,取疱疹下或疼痛区皮肤组织,HE染色及S-100染色下观察皮损区组织病理切片中淋巴细胞异常浸润及神经末梢损害情况。②临床研究部分:皮神经阻滞组18例(n=18),应用腺苷钴胺0.5 mg+甲强龙40 mg+0.3%利多卡因,总量约10～15 ml,于皮肤损伤最严重或感觉疼痛最明显的区域,自神经分布近端至远端进行皮下阻滞。药物治疗组18例(n=18),常规口服抗病毒药物+维生素B12+镇痛药物,并且在皮损区辅助应用外用药物。评价疗效。结果①基础研究部分:活体中带状疱疹受累处皮肤存在中重度免疫炎性反应。②临床研究部分:皮神经阻滞组的结痂时间较药物治疗组缩短,疼痛缓解程度较药物治疗组明显,且差异具有统计学意义(P<0.05)。结论①在带状疱疹病毒受累皮肤区,周围神经周围有大量的免疫细胞浸润,神经轴突的完整性遭到破坏,存在免疫炎性反应,这是带状疱疹产生疼痛的重要病理学因素。②皮神经阻滞治疗方法可以通过有效的抑制带状疱疹皮损区免疫炎性反应,更快的促进局部皮损结痂愈合且明显减低带状疱受累区的疼痛程度。

皮肤扩张术在面部皮肤病损修复中的应用

目的:总结应用皮肤扩张术修复面部皮肤病损的临床经验。方法:Ⅰ期手术选用50～400ml容量的扩张器埋植于面部病损邻近皮肤正常区域的皮下层,注水扩张1～4个月;Ⅱ期切除面部病损,运用扩张皮瓣修复皮肤缺损。结果:23例中,21例面部病损切除后利用扩张皮肤形成局部皮瓣一次完成修复,修复后的皮肤质地和色泽与受区接近,外形满意。一次修复缺损创面最大者约8cm×11cm。1例于Ⅰ期手术后53天扩张器外露,提前取出扩张器形成局部皮瓣加植皮修复,效果良好;1例为较大面积的面部扁平瘢痕,第一次扩张后仍残留1/4瘢痕,经二次扩张遂完全切除瘢痕并修复。结论:运用皮肤扩张术修复面部病损切除后的皮肤缺损是一种较为理想的修复方法,在某些特殊情况下,可能还需要辅助植皮或应用连续扩张法完成修复。

皮肤良恶性病变DNA原位定量AgNORs的图象分析

应用彩色病理图象分析仪对６１例皮肤良恶性病变进行ＤＮＡ原位定量及ＡｇＮＯＲｓ的图象分析，结果表明，皮肤癌的ＤＮＡ倍体多呈现Ｃ５、＞Ｃ５，ＡｇＮＯＲｓ的图象分析示３种变量即ＧＮ（颗粒数目）、ＧＡ（颗粒面积）、ＧＶ（颗粒的变异系数）意义较大，在良恶性皮肤病中差异非常显著（Ｐ＜０．０１）。研究表明，ＤＮＡ原位定量与ＡｇＮＯＲｓ的图象分析对判断皮肤良恶性病变。

Q开关ND:YAG激光治疗皮肤色素性损害的疗效分析

目的 观察Q开关ND :YAG激光治疗皮肤色素性损害的临床疗效与治愈次数和不良反应。方法 用Q开关ND :YAG激光 ( 10 64nm、 5 3 2nm、 75 5nm) ,针对不同病变类型及病人个体差异 ,采用不同波长和参数治疗 2 3 80例皮肤色素性损害 ,包括太田痣、雀斑样痣、咖啡斑、毛痣、老年斑、雀斑、文身、文眉、文眼线、文唇线。结果 治疗文身、太田痣、文眉、雀斑、文唇线、老年斑有效率均达到 82 %以上 ,其中文身、太田痣效果最好 ,有效率达到 90 %以上。痊愈率中太田痣最高 ,达到 86 3 % ,其次为文眉、雀斑、老年斑、蓝黑色文身、红色文身及文唇线和文眼线。结论 Q开关ND :YAG激光对文身、太田痣、文眉、雀斑、文唇线、老年斑及文眼线效果好 ,对雀斑样痣及咖啡斑效果略差。

克拉玛依地区28例皮肤利什曼病的临床病理观察

本文对克拉玛依地区查见的２８例病期为１—４８个月的皮肤利什曼病人的皮肤损害进行了临床表现与病理变化的观察。皮肤的表现一般可分为丘疹或斑块、软性结节或脓肿、溃疡及硬性结节４种临床类型。相应的组织病变，在丘疹或斑块型，主要为原虫引起的巨噬细胞聚集及炎性反应，软性结节或脓肿及溃疡型则为原虫大量繁殖导致的组织坏死液化及脱落，硬性结节主要是受损组织的修复与增生及原虫数的大量减少。以上结果，对了解皮肤利什曼病的病理演变过程以及与皮肤型黑热病的鉴别诊断均有一定帮助。

色素痣样皮损381例的临床与组织病理分析

目的:探讨381例色素痣样皮损的临床与病理之间的关系。方法:对381例色素痣样的病例做临床及组织病理诊断回顾性分析。结果:381例色素痣样皮损中,病理诊断符合色素痣327例,符合率为93.7%,组织病理类型:皮内痣219例(67%),混合痣89例(27.2%),交界痣8例(2.4%)及其他11例(3.4%);327例色素痣中,先天性色痣140例(42.8%),组织病理类型:皮内痣74例(52.9%),混合痣55例(39.3%),交界痣7例(5%)及蓝痣4例(2.8%);临床误诊为色素痣54例,病理诊断居前5位为脂溢性角化病、血管角皮瘤、基底细胞癌、黑素瘤、鳞状细胞癌。结论:色素痣样皮损的正确诊断需要结合组织病理分析。

81例CT引导下经皮肺穿刺活检标本临床病理结果

目的:分析CT引导下经皮肺穿刺活检标本的临床病理学及免疫组织化学诊断,结合血清肿瘤标志物检测探讨其对肺部占位性病变的诊断价值。方法:回顾性分析81例肺部占位性病变患者CT引导下肺穿刺活检标本病理学、25例免疫组织化学诊断及73例血清肿瘤标志物癌胚抗原(carcinoembr yonic antigen,CEA),细胞角蛋白19片段(cytokeratin-19-fragment,CYFR A21-1),神经元特异性烯醇化酶(neuron-specific enolase,NSE)检测结果。结果:81例CT引导下肺穿刺活检标本组织病理学诊断率为96.3%,其中恶性病变53例(腺癌29例,鳞癌20例,小细胞癌1例,未明确类型的非小细胞肺癌1例,其他类型肺癌1例,胸腺恶性肿瘤1例);良性病变25例(炎性包块7例,结核5例,肺炎7例,硅沉着病3例,炎性假瘤2例,神经鞘瘤1例);25例行免疫组织化学检查的病例中诊断恶性肿瘤24例(腺癌13例,鳞癌8例,小细胞癌1例,未确定分型2例),其中P63及CK5/6在鳞癌阳性率为87.5%,TTF-1及CK-7在腺癌阳性率为69.2%和76.9%,差异有统计学意义(P<0.05);CEA,CYFR A21-1,NSE及3项联合对肺癌诊断的灵敏度分别为54.9%,68.6%,37.3%,80.4%。结论:CT引导下经皮肺穿刺活检标本病理诊断阳性率高,结合免疫组织化学及血清肿瘤标志物联合检测可为肺癌的诊断及病理分型提供辅助性参考依据。

先天性厚甲症伴多发性皮角1例

患者男,18岁。甲及掌跖部皮损18年。皮损特征表现为:20甲高度增厚、掌跖部严重角化、毛囊角化,尤以肘膝部明显、乳头角化及面部多发性皮角样损害。皮肤活检示:表皮高度角化过度及灶性角化不全,真皮少量炎症细胞浸润。诊断:先天性厚甲症;多发性皮角。

颜面部皮肤炭疽9例的诊断与治疗

目的观察颜面部皮肤炭疽的临床特征及其诊断和治疗。方法对收治的9例颜面部皮肤炭疽的临床表现及诊治方法进行分析和总结。结果 9例患者均有流行病学接触史;症状表现为病变部位最初出现红色丘疹,2～3天出现水疱,周围组织水肿,水疱破裂后出现局部组织坏死。其中7例形成典型的1～3 cm直径大小的黑色焦痂。病变部位分泌物直接涂片镜检均可见革兰阳性粗大杆菌。9例患者均给予青霉素钠静脉滴注,并高锰酸钾溶液局部湿敷,其中5例联用乳酸左旋氧氟沙星静脉滴注,3～6周后均痊愈。结论根据流行病学接触史、典型的临床表现,结合病原学检查可对颜面部皮肤炭疽做出明确的诊断,临床治疗药物首选青霉素。

先天性脊柱侧凸患者的背部皮肤病变及其临床意义

目的分析先天性脊柱侧凸患者背部中线处皮肤病变及其临床意义。方法回顾性分析在我院初次诊治、存在背部中线处皮肤病变的先天性脊柱侧凸,除外脊髓脊膜膨出患者。结果在1990年～2005年诊治的先天性脊柱侧凸患者中,63例患者存在背部中线处皮肤病变,具体表现为背部异常毛发32例,皮肤色素斑22例,背部软组织包块3例,背部皮肤凹陷1例,色素斑和异常毛发3例,异常毛发和皮下结节2例。13例(20.6%)患者同时存在椎管内异常,包括脊髓纵裂10例,脊髓空洞症2例,脂肪瘤1例。13例患者中,先天性脊柱侧凸类型表现为形成障碍3例,分节障碍2例,混合型8例,84.6%(11/13)为多发椎体畸形。此13例患者的皮肤异常具体表现为多毛7例,色素斑3例,背部肿物2例,皮肤凹陷1例,不同皮肤异常类型之间椎管内异常的发生率比较无统计学意义。同期诊治无背部皮肤病变的先天性脊柱侧凸294例,11例(3.7%)存在椎管内异常,其发生率同存在皮肤病变的患者相比,差异存在统计学意义(P=0.000)。结论背部中线处皮肤病变可能是先天性脊柱侧凸患者尤其是合并多发椎体畸形者合并椎管内异常的一个标志。

48例结节性硬化患者皮损临床特点分析

目的探讨结节性硬化皮损的特点。方法收集48例结节性硬化患者皮损特点并进行分析。结果血管纤维瘤的发生率为79.2%,分布于面中部者100%,其中2～10岁为高发年龄。叶状脱色斑发生率为66.7%,0～2岁为高发年龄。鲨鱼皮样斑发生率为54.2%,92.3%分布于腰骶、后背部位,2～10岁为高发年龄。甲周纤维瘤发生率为31.3%,分布于甲根,11～19岁为高发年龄。结论结节性硬化皮损发生率高而且复杂,多在幼年发病。

冠脉严重钙化病变旋磨治疗的临床观察

目的评价冠脉旋磨治疗严重钙化病变的临床疗效观察。方法 24例患者34处严重钙化病变行旋磨治疗。观察治疗的即刻成功率及随访结果。结果所有患者均成功进行了旋磨治疗及药物洗脱支架植入,手术成功率100%。2组患者全部手术成功,术中无死亡、心肌梗死和冠脉穿孔等并发症发生。15例患者(62.5%)完成了9个月造影复查,无明显支架内再狭窄。结论冠状旋磨治疗可安全用于冠状动脉严重钙化病变,提高介入治疗成功率。