Diagnostic and management challenges in primary cutaneous anaplastic large cell lymphoma with necrosis,inflammation,and surgical intervention:A case report

BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)poses significant diagnostic difficulties due to its similarity in the appearance of skin lesions with chronic inflammatory disorders and other dermatological conditions.This study aims to investigate these challenges by conducting a comprehensive analysis of a case presenting with PC-ALCL,emphasizing the necessity of accurate differentiation for appropriate management.CASE SUMMARY An 89-year-old female patient with diabetes and hypertension presented with arm and abdominal ulcerated mass lesions.Diagnostic procedures included skin biopsies,histopathological assessments,and immunohistochemistry,complemented by advanced imaging techniques to confirm the diagnosis.The patient’s lesions were determined as PC-ALCL,characterized by necrosis,chronic inflammation,and a distinct immunophenotypic profile,including CD30,CD3,CD4,and EBER,CD56,MUM-1,Ki 67-positive in>80%of tumor cells,CD10,but negative for anaplastic lymphoma kinase,CD5,CD20,PAX-5,Bcl-2,Bcl-6,CD8,and CD15.Recurrence was not reported at the 6-month follow-up.CONCLUSION Accurate PC-ALCL differentiation from similar conditions is crucial for effective management and requires a multidisciplinary approach.

Topical halometasone cream combined with fire needle pretreatment for treatment of primary cutaneous amyloidosis:Two case reports

BACKGROUND Primary cutaneous amyloidosis(PCA)is a chronic metabolic skin disease that has a detrimental impact on physical and mental health.It appears as mossy papules and severe itching,which is long-term and recurrent.Traditional treatments are unsatisfactory,especially for refractory cases.Fire needle therapy,which is widely used in China,has shown good clinical efficacy,as well as advantages concerning safety and cost.Clinical reports about fire needle treatment of this disease are few at present.CASE SUMMARY We report two older men who had developed maculopapules with itchiness on the trunk and arms for more than 10-15 years.Due to the dermatopathological findings,PCA was our primary consideration.They received topical halometasone cream and pretreatment with fire needle for 8-16 wk.Both patients showed significant improvement of lesions.Neither patient had recurrence with a minimum of 2 years of follow-up.CONCLUSION Topical halometasone cream and pretreatment with fire needle could be a fast,safe,and economic treatment for PCA.

T-CELL RECEPTOR GENE REARRANGEMENT ANALYSIS IN THE PRIMARY CUTANEOUS T-CELL LYMPHOMA

Object: The present paper is to evaluate the significance of T cell receptor (TCR) gene rearrange ments in primary cutaneous T cell lymphomas (PCTCL) as detected by analysis of Southern Blot (SBA) and polymerase chain reaction (PCR). Patients and Methods: Skin specimens and peripheral blood samples were taken from 44 patients with PCTCL, including 30 patients with mycosis fungoides (MF), 2 patients with Sezary's syndrome (SS), and 12 patients with PCTCL other than MF and SS (PNCTCL). 11 patients with a presumptive diagnosis of MF, 23 patients with lymphoproliferative dermatoses including lymphomatoid papulosis (LyP) and 8 patients with benign cutaneous lymphoid infiltrates were simultaneously studied by the amplification of junctional V (variable) J (joining) sequences of the rearranged TCRγ genes by PCR(TCRγPCR) and the analysis of TCRb chain genes by SBA(TCRβSBA) for detection of clonal gene rearrangements (GR). One lymph node specimen of a case with MF IIA was also detected by TCRγ PCR and TCRβSBA. Results: In MF, GR were detected by TCRγPCR and TCRβSBAb in 83.3 85.7% and 66.7% 71.4% of skin specimens of cases IIA IIB and in 57.1% 70.0% and 14.3% 10.0% of those of cases IA IB, respectively. GR were seen in 66.7% 71.4% and 33.3% 43.0.% of blood samples of cases IIA IIB, and 42.9% 40.0% and 0 10.0% of those of cases IA IB, respectively. GR was confirmed by TCRγ PCR and TCRβSBA in one lymph node showing dermato pathic lymphadenopathy of a case with MF IIA. In 11 patients of clinically suspected MF, GR were present in skin specimens of 5 cases (45.4%) and in blood samples of 3 cases ( 27.3% ) by TCRγ PCR. In PNCTCL, GR were found in 9 skin specimens (90.0%) from 10 patients detected by TCRγ PCR and in 6 skin specimens (75.0%) from 8 patients detected by TCRβSBA. GR were also seen in 6 blood samples (72.8%) from 11 patients detected by TCRγ PCR, and in 7 blood samples (70.0%) from 10 patients by TCRβSBA. In SS and LyP, GR were detected by TCRγ PCR and TCRβSBA in each of the two skin specimens of two cases with LyP and in each of the two blood samples of two cases with SS. GR were seen in one skin specimen of one case with SS and one blood sample of one case with LyP detected by TCRγPCR. Conclusions: This study demonstrated that TCRγ PCR is a rapid, more sensitive tool than TCRβSBA, can be used in the analysis of T cell clonality in skin, lymph node and blood samples of patients with PCTCL and indicated that this method forms a useful supplement to other methods for diagnosis of early and suspected MF, confirmation of PNCTCL and determination of extracutaneous involvement of lymph node and blood.

Chidamide combined with traditional chemotherapy for primary cutaneous aggressive epidermotropic CD8+cytotoxic T-cell lymphoma:A case report

BACKGROUND Traditional chemotherapy has benefited many patients with non-Hodgkin's lymphoma,but results in a very poor response in patients with rare lymphomas or refractory lymphomas.Previous studies have shown that chidamide has potential anti-lymphoma activity and reverses lymphoma cell chemoresistance to increase the chemosensitivity of lymphoma cells to traditional chemotherapy.CASE SUMMARY A 14-year-old boy was admitted to our hospital with a 5-d history of generalized erythema,papules,and blisters.Initially,the disease was refractory to potent antiallergic and anti-infective treatment,and his condition progressively worsened.Skin biopsy revealed primary cutaneous aggressive epidermotropic CD8+cytotoxic T-cell lymphoma.Considering that the disease is extremely rare in clinical practice,existing case reports have shown poor efficacy with traditional chemotherapy alone.We recommend chidamide combined with traditional chemotherapy for treatment.The regimen was as follows:Chidamide 30 mg/biw,cyclophosphamide 1100 mg/d1,pirarubicin 70 mg/d1,vincristine 2 mg/d1,dexamethasone 20 mg/d1-5,etoposide 100 mg/d1-5,in a 21 d cycle.The treatment effect was considerable,and complete remission was achieved after 4 cycles of treatment,after which the patient completed a total of 6 cycles of treatment.Subsequently,the patient regularly took chidamide 20 mg/biw as maintenance therapy for 1 year.To date,the patient has been disease-free for 3 years.CONCLUSION This case suggests that the combination of chidamide and traditional chemotherapy is effective in primary cutaneous aggressive epidermotropic CD8+cytotoxic T-cell lymphoma.

Primary Cutaneous Apocrine Carcinoma of the Neck Involving the Parotid Gland: A Case Report and Review of the Literature

Introduction: Primary cutaneous apocrine carcinoma (PCAC) is a rare skin malignant tumor that originates from areas with a high concentration of apocrine glands. The incidence of PCAC in the neck is relatively low. The age of onset in PCAC ranges from 5 to 70 years old. Clinically, PCAC typically shows up as painless nodules or lumps. In immunohistochemistry, positive expression of CK-7, GCDFP-15, AR, and myoepithelial markers are helpful in the diagnosis of PCAC. This case report pertains to a 59-year-old male of Asian descent. Five years ago, a painless mass was discovered behind his right ear, which grew slowly. This patient was identified as PCAC based on clinical symptoms, pathology, immunohistochemistry, and imaging characteristics. PCAC involved the parotid gland in this case;it is easy to be misdiagnosed as a primary malignant tumor of the parotid gland on imaging. Furthermore, a thorough set of clinical, imaging, pathological, and immunohistochemical examinations must be performed to make a diagnosis because it is challenging to differentiate PCAC from metastatic breast carcinoma. Conclusion: A well-developed multidisciplinary examination is essential because PCAC can be challenging to diagnose and differentiate.

Primary cutaneous mantle cell lymphoma:Report of a rare case

BACKGROUND We describe the case of a 74-year-old man diagnosed with primary cutaneous mantle cell lymphoma(MCL),an extremely rare and controversial condition that is not included in the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas.CASE SUMMARY The patient presented diffuse cutaneous erythematous plaques and nodules throughout the body.Skin lesions were biopsied and histopathological examination showed diffuse monomorphic lymphocyte infiltration in the dermal and subcutaneous layers,sparing the epidermis.Immunohistochemical staining revealed CD20,cyclin-D1,CD5,and SOX-11 expression.Fluorescence in situ hybridization showed CCND1/IGH gene rearrangement.Correct diagnosis of primary cutaneous MCL requires ensuring that no other parts are involved;these cases require close follow-up to monitor their possible progression to systemic disease and for treating relapsed cutaneous disease.In this case,positron emission tomography scanning and clinical staging revealed no systemic involvement,and follow-up examination at 20 mo after diagnosis showed no evidence of systemic disease.The prognosis of primary cutaneous MCL is relatively good.Our patient received six cycles of chemotherapy,and the cutaneous manifestations presented almost complete remission.CONCLUSION Primary cutaneous MCL is rare,and its prognosis is relatively favorable.However,correct diagnosis is a prerequisite for proper treatment.

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.

Primary cutaneous B cell lymphoma: Clinical features, diagnosis and treatment

Primary cutaneous B cell lymphoma(PCBCL) is defined as B cell lymphomas that presents in the skin without any evidence of extra-cutaneous involvement at diagnosis. They are the second most common type of primary cutaneous lymphomas accounting for 25%-30%. Since the prognosis and treatment differ from systemic lymphomas involving the skin, differential diagnosis is very important. PCBCL is a heterogeneous group of disease comprising different B cell lymphomas with distinct treatment and prognosis. PCBCL is divided into 5 subclasses according to World Health Organization and European Organization of Research and Treatment of Cancer classification. Primary cutaneous marginal zone lymphoma and primary cutaneous follicle centerlymphoma are indolent forms and often confined to skin at presentation and during the course of the disease. But primary cutaneous diffuse large B cell lymphoma, leg type and intravascular large B cell lymphoma are more aggressive forms that may disseminate to extra-cutaneous tissues. There is not a treatment consensus since they are rare entities. Local therapies like radiotherapy, surgery or intralesional steroids are options for localized disease in indolent forms. More disseminated disease may be treated with a systemic therapy like single agent rituximab. However combination chemotherapies which are used in systemic lymphomas are also required for aggressive PCBCL. Although indolent forms have relatively better prognosis, early relapses and disseminated diseases are mostly observed in aggressive form with a consequent poor prognosis.

Relative frequency and survival of primary cutaneous lymphomas： a retrospective analysis of 98 patients

Background The World Health Organization and European Organization for Research and Treatment of Cancer （WHOEORTC） classification in 2005 promoted the comparisons of primary cutaneous lymphoma （PCL） subtypes between different countries.The relative frequency of PCL varied according to geography.The study aimed to analyze the relative frequency and survival of PCLs in China and to compare the data with the published results from other countries.Methods We analyzed 98 patients with PCLs over a 6-year period and reclassified them according to the most recent WHO-EORTC classification （2005）.Disease-specific survival rate and curves according to specific subtypes such as mycosis fungoides,lymphomatoid papulosis,and primary cutaneous peripheral T-cell lymphoma,unspecified was also calculated.Results The relative rate of PCL in China was distinct from those in Western countries.Our study showed a higher frequency of cutaneous T-and NK-cell lymphomas （CTCLs） （94%）,and a lower frequency of cutaneous B-cell lymphomas （CBCLs） （6%）.The 5-year survival rate of the total PCLs was 82%.There was no significant difference in the 5-year survival rate （P 〉0.05 by Log-rank test） between CTCL （80%） and CBCL （100%）.Conclusions The higher percentage of CTCL in China may provide a clue to further study the etiological factors of PCLs.Racial variations in factors such as HLA determinants may play a role in the development of CTCL.

Cutaneous B cell lymphoma presenting as a soft tissue swelling

Cutaneous lymphomas are classified as B cell and T cell lymphomas which are seen very rarely. They present as papules or nodules in the absence of any extracutaneous involvement. We present a patient who had a diffuse cutaneous B cell lymphoma and review literature of this uncommon tumour.

巴西诺卡菌感染所致的原发性皮肤诺卡菌病一例

原发性皮肤诺卡菌病是一种由诺卡菌属直接感染皮肤及皮下组织引起的少见感染性疾病,临床表现多样,极易被误诊。本例患者左前臂红斑、结节、脓肿及溃疡,表面大量脓性分泌物2周。活检组织培养长出黄白色菌落,鉴定为巴西诺卡菌。患者口服复方磺胺甲噁唑治疗50天后痊愈。

原发性皮肤边缘区淋巴瘤1例

患者男,41岁,背部出现皮损,不伴痛痒6个月余。皮肤科情况:背部可见一直径约1.0 cm红色、近圆形、表面光滑的结节,周围有红斑晕,质地略硬,无触压痛。组织病理检查:肿瘤细胞在真皮内弥漫性浸润,呈现上方浸润多且范围广,下方浸润少且范围窄,伴有反应性生发中心;可见“三相”模式,即中央为淡染的生发中心,绕以深染的滤泡套细胞,以及淡染的肿瘤性边缘区细胞;并可见较多浆细胞、淋巴浆细胞样细胞、边缘区细胞(较大且淡染的细胞)以及小的反应性淋巴细胞,偶有大的母细胞。免疫组化染色:肿瘤性边缘区细胞CD20、CD79a、B细胞淋巴瘤-2(Bcl-2)、多发性骨髓瘤癌基因(MUM-1)、CD43均阳性,Kappa(浆样细胞阴性),Lambda(浆样细胞阳性),Ki-67达25%;CD3、CD21、Bcl-6均阴性。分子病理检测结果示T淋巴细胞克隆性基因重排检测阴性,B细胞淋巴瘤克隆性基因重排检测阳性。诊断:原发性皮肤边缘区淋巴瘤。

Marginal zone lymphoma with severe rashes: A case report

BACKGROUND Marginal zone lymphoma(MZL)is an indolent subtype of non-Hodgkin lymphoma(NHL),which is rare clinically with severe rashes as the initial symptom.CASE SUMMARY This study reports a case of MZL with generalized skin rashes accompanied by pruritus and purulent discharge.First-line treatment with rituximab combined with zanubrutinib had poor effects.However,after switching to obinutuzumab combined with zanubrutinib,the case was alleviated,and the rashes disappeared.CONCLUSION For patients with advanced stage MZL not benefiting from type I anti-CD20 monoclonal antibody(mAb)combination therapy,switching to a type II anti-CD20 mAb combination regimen may be considered.This approach may provide a new perspective in the treatment of MZL.

原发性皮肤淀粉样变的治疗研究进展

原发性皮肤淀粉样变是一种慢性瘙痒性皮肤病,常给患者的日常生活造成较大困扰。该病的治疗方法目前尚无统一标准,现有的治疗手段主要包括药物治疗、物理治疗及手术治疗三大类。然而,治疗方案的选择需依据患者症状的严重程度、个体差异及医师的临床经验来综合决定。随着研究的深入,新的治疗方法不断涌现,患者在治疗过程中需与医生密切配合,及时调整治疗方案。在制定个体化的治疗计划时,全面考虑患者的具体情况及长期管理是至关重要的。

原发性皮肤黏液癌4例临床病理分析

目的:探讨原发性皮肤黏液癌临床病理学特点及预后。方法:收集4例患者临床及组织病理资料,并进行分析。结果:肿瘤位于真皮层内,呈腺管状、筛状或实体状排列的肿瘤细胞漂浮于黏液湖中。例2少许腺管外周可见肌上皮细胞包绕。部分肿瘤细胞呈靴钉样排列于巢团周,内部呈圆形或卵圆形,细胞异形明显,可见核仁及较多核分裂。例2 P63、细胞角蛋白(CK)5/6及钙调蛋白(calponin)阳性,例3散在表达calponin。例2及例4增殖细胞核抗原(Ki-67)高表达。4例均表达大疱病液蛋白(GCDFP)-15、乳珠蛋白(mammoglobin)、GATA结合蛋白(GATA)-3、CK7、雌激素受体(ER)、孕激素受体(PR)、雄激素受体(AR)、MUC1及MUC2。例1多次复发。结论:原发性皮肤黏液癌较为罕见,好发于眼周。肌上皮标志物阳性提示原位病变的存在,预后较好。

基于中国诊疗现状的《NCCN指南:原发性皮肤淋巴瘤(2024年第1版)》解读

原发性皮肤淋巴瘤是一组异质性疾病,具有独特的临床表现及组织学特征,部分亚型的诊断与治疗存在挑战。2023年12月美国国立综合癌症网络(National Comprehensive Cancer Network,NCCN)更新发布了《NCCN指南:原发性皮肤淋巴瘤(2024年第1版)》,与2023年版指南比较,此次更新主要集中于诊断评估原则、分期标准、不同分期蕈样肉芽肿的治疗原则及对放疗的建议等方面。本文结合我国原发性皮肤淋巴瘤的诊疗现状及特点,对该指南的主要推荐内容进行解读,以促进其在我国临床实践中的应用。

基于lncRNA PVT1表达分析银杏内酯B对ITP患者B淋巴细胞及Treg/Th17免疫失衡的影响

目的探讨银杏内酯B对原发性免疫血小板减少症(ITP)患者长链非编码RNA浆细胞瘤转化迁移基因1(lncRNA PVT1)、B淋巴细胞和调节性T淋巴细胞/辅助性T淋巴细胞17(Treg/Th17)免疫失衡的影响及其相关性。方法依据双盲法将2021年5月至2023年5月廊坊市中医医院收治的130例ITP患者分为对照组(65例)和观察组(65例)。对照组给予长春地辛治疗,观察组给予银杏内酯B治疗,两组均治疗4周。比较两组患者临床疗效;比较两组患者治疗前后lncRNA PVT1表达、B淋巴细胞亚群[CD19^(+)、B1淋巴细胞]比例、Treg/Th17免疫失衡细胞因子[白细胞介素-17(IL-17)、白细胞介素-21(IL-21)];分析lncRNA PVT1表达与CD19^(+)、B1淋巴细胞比例以及IL-17、IL-21水平的相关性。结果研究过程中对照组失访7例,观察组失访5例,最终分组为对照组58例,观察组60例。治疗后两组患者lncRNA PVT1表达、IL-17、IL-21水平以及CD19^(+)、B1淋巴细胞比例较治疗前均有所降低(P<0.05),观察组治疗后lncRNA PVT1表达、IL-17、IL-21水平以及CD19^(+)、B1淋巴细胞比例明显低于对照组(P<0.05)。与对照组相比,观察组临床疗效更优(P<0.05)。lncRNA PVT1表达与CD19^(+)、B1淋巴细胞、IL-17、IL-21水平均呈正相关(P<0.05)。结论银杏内酯B对ITP患者具有较高临床疗效,可以显著改善其lncRNA PVT1表达、B淋巴细胞比例、Treg/Th17免疫失衡细胞因子水平,值得临床推广。

原发性皮肤浆细胞瘤1例

报告1例原发性皮肤浆细胞瘤。患者男,76岁。初发为颈部单发的无痛性结节,后发展为多发损害,组织病理检查及免疫组化染色结果提示真皮全层及皮下脂肪克隆性浆细胞增生。患者病程近10年,长期随访及多次系统检查未发现系统受累症状,诊断为原发性皮肤浆细胞瘤。给予MP(M:马法仑,P:泼尼松)方案化疗,取得较好的效果。

多变根毛霉引起原发皮肤毛霉病1例

报告1例由多变根毛霉引起的皮肤毛霉病。患者男,33岁。右上肢外伤后弥漫性斑块伴肿胀、化脓7年余。免疫功能检测CD4+T细胞略低(28%),皮损组织病理检查示真皮中下层有炎细胞及多核巨细胞浸润,并见粗大较短的无隔菌丝。经真菌培养菌种鉴定为多变根毛霉。患者曾接受伊曲康唑、特比萘芬和氟康唑等抗真菌药物治疗,均无满意疗效,最后经用两性霉素B治疗痊愈,随访6个月未复发。

CD8^＋的淋巴瘤样丘疹病1例

患者女,38岁。全身反复红斑、斑片8年,丘疹、结节1年。全身多处泛发色素沉着斑及红色小丘疹,左腰部有一直径1cm大小的斑块。斑块处组织病理示:真皮及皮下弥漫大细胞,有异形性;免疫组化:异形细胞LCA(+)、CD3(+)、CD4(+)、CD8(+)、CD30(+)、GranzymeB(+),诊断为淋巴瘤样丘疹病,A型。该患者以红斑起病之后出现丘疹,但从未出现溃疡坏死,丘疹的大小和可自行消退的特点符合淋巴瘤样丘疹病,CD8+是LyP较为少见的免疫表型。该病应与皮肤CD30(+)间变性大细胞淋巴瘤和蕈样肉芽肿鉴别,需监测其预后及转归。