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.

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.

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.

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 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.

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.

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.

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.

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。

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。

原发性皮肤弥漫大B细胞淋巴瘤，腿型

报告1例原发性皮肤弥漫性大B细胞淋巴瘤(腿型)。患者女,82岁。左小腿出现3个红色结节2个月,组织病理检查发现真皮内淋巴样细胞浸润,无嗜表皮现象,细胞体积大,肿瘤细胞CD20(+),CD79α(+),Bcl-2(+),Bcl-6(+),Ki-67 70%(+),MUM-1(+),Pax-5(+),CD10(-),诊断为原发性皮肤弥漫性大B细胞淋巴瘤(腿型),全身检查未发现皮肤以外系统受累证据,行局部肿瘤切除及口服糖皮质激素治疗。

原发皮肤间变大细胞淋巴瘤临床治疗探讨

目的：探讨原发皮肤间变大细胞淋巴瘤（primary cutaneous anaplastic large cell lymphoma，PCALCL）的临床特征及治疗方法。方法：回顾性分析天津医科大学肿瘤医院2009年1月至2012年12月收治的6例PCALCL患者的临床资料。结果：中位发病年龄为54（38～60）岁，男女各3例；单发皮损3例，多发皮损3例。患者初期出现红斑或红色片状皮下结节样皮损，结节多高于皮面，界清、质硬，多不伴疼痛，部分患者伴皮损处瘙痒，结节逐渐增大，多发者相邻皮损可出现融合，部分皮损可表现为自限性或反复出现，搔抓后伴脱屑，随着病情发展，皮损颜色由红变暗，皮损表面可破渍。仅1例出现皮肤外浅表淋巴结侵犯。3例单发皮损患者完全手术切除后行辅助化疗和（或）放疗，均无病生存。3例多发皮损患者以化疗为主，2例出现复发，1例无病生存。中位随访时间24（11～35）个月，无进展生存率为66．7％，总生存率100％。结论：对于单发局限病灶，手术切除或放射治疗就可以获得满意的控制率。化疗可试用于控制多发病灶及有真皮外侵犯的患者。

原发性皮肤弥漫性大B细胞性淋巴瘤(腿型)17例临床病理分析

目的探讨原发性皮肤弥漫性大B细胞性淋巴瘤(腿型)(PCDLBCLLT)的临床病理特点。方法回顾性分析17例PCDLBCLLT的临床资料、组织学形态和免疫组化标记。结果 17例PCDLBCLLT的发病年龄为31～86岁,平均64.4岁;其中男性9例,女性8例,男女之比为1.1∶1;主要发生于腿部和躯干部。组织学表现为弥漫分布的肿瘤细胞,以中心母细胞和免疫母细胞为主,核分裂象易见,肿瘤组织不累及表皮。免疫组化:肿瘤细胞表达B细胞相关抗原,bcl-2、MUM1、FOX-P1和bcl-6(+),Ki-67增殖指数为60%～90%。结论 PCDLBCLLT是一种独特类型的大B细胞性淋巴瘤,预后较差。

发生于阴囊的原发性皮肤间变性大细胞淋巴瘤

报告1例以阴囊多发结节斑块为表现的原发性皮肤间变性大细胞淋巴瘤。患者男,53岁。因阴囊结节6个月就诊。体格检查:阴囊多发暗红色结节斑块,伴溃疡形成,双侧腹股沟淋巴结肿大。皮损组织病理学检查示真皮弥漫淋巴样细胞浸润,瘤细胞偏大,核不规则;免疫病理检查示CD30(+)、CD8(+)、间变型淋巴瘤激酶(ALK)-l(-)。复习相关文献,发生于生殖器部位的原发性皮肤间变性大细胞淋巴瘤国内未见报道,英文文献中仅有2例报道。

原发性皮肤CD30^+间变性大细胞淋巴瘤1例

报告1例原发性皮肤CD30+间变性大细胞淋巴瘤。患者男,72岁。右足拇趾红肿,外侧有一2.0cm×2.5cm红色溃疡面,伴血性液体渗出,近端有一1.5cm×2.0cm暗红色结节,表面结痂。皮损组织病理及免疫组化标记确诊为原发性皮肤CD30+间变性大细胞淋巴瘤。

原发性皮肤CD4^＋多形性小／中T细胞淋巴瘤1例临床病理观察并文献复习

报告1例原发性皮肤CD4+多形性小/中T细胞淋巴瘤。患者女,45岁。右膝右上方反复红斑、结节15年。组织病理检查示真皮全层及皮下脂肪层弥漫性结节性致密小到中等大淋巴样细胞浸润。细胞有异形,其间混杂少量炎性细胞,无亲表皮现象。免疫组化检查示全T抗原缺失的Th表型。诊断:原发性皮肤CD4+多形性小/中T细胞淋巴瘤。

原发性皮肤边缘区B细胞淋巴瘤临床病理特征

目的探讨原发皮肤边缘区B细胞淋巴瘤(PCMZL)的临床和病理学特征,诊断和鉴别诊断。方法对2例PCMZL和1例皮肤淋巴组织增生(CLP)病例进行临床特征、组织形态学、免疫表型及PCRIgH基因重排分析。结果免疫组化示2例PCMZL的小淋巴细胞CD20和CD79α(),bcl-2、BOB.1和Oct-2(),MUM-1(+),CD5、CD10、bcl-6和CD23(-);浆细胞CD138、MUM-1(),限制性表达轻链kappa。1例CLP的小淋巴细胞CD20、CD3和bcl-2(),MUM1(+);浆细胞同时表达κ及λ。IgH扩增2例PCMZL呈单克隆性,1例CLP显示多克隆性。结论PCMZL属MALT-L家族,形态学上肿瘤细胞由异源性小淋巴细胞组成,免疫学上显示后生发中心标记。浆细胞轻链限制性和B细胞抗原受体基因克隆性重排对诊断有非常重要的帮助。

泛发性原发皮肤 CD30^+间变性大细胞淋巴瘤

报告1例泛发性原发皮肤CD30^+间变性大细胞淋巴瘤。患者男,44岁。因全身皮肤多发红斑、结节、溃疡50余天就诊,皮损组织病理及免疫组化检查结果提示间变性大细胞淋巴瘤,免疫组化检查:瘤细胞CD3(++),CD30(++),Ki-67约50%(+),CD20、Ckpan、S-100蛋白、HMB45、间变性淋巴瘤激酶(ALK)-1均阴性,诊断为间变性大细胞淋巴瘤。

自体外周血造血干细胞移植治疗原发皮肤γδT细胞淋巴瘤

目的:探讨原发皮肤γδT细胞淋巴瘤的临床特点和治疗方法。方法:对在2013年7月我科确诊的1例原发皮肤γδT细胞淋巴瘤患者的临床资料和治疗经过进行分析。患者系39岁女性,以多发皮下结节为主要临床特点,皮肤活检病理检查免疫表型为CD20^-、CD3^+、CD4^-、CD8^-、CD56^+、TIA^-1^+、Ki^-67^+(约60%);浆细胞κ^+(部分)/λ^+(部分);组织细胞CD4^+、CD68/PGM1^+;βF1^-、EB病毒EBER原位杂交检查阴性。结果:给予包含左旋门冬酰胺酶的化疗方案获得疾病缓解,于2014年2月行自体造血干细胞移植术,术后14 d粒细胞稳定植入,12 d血小板稳定植入。术后随访,缓解至今。结论:原发皮肤γδT细胞淋巴瘤临床罕见,容易误诊,疾病呈侵袭性,临床进展快,应用含左旋门冬酰胺酶的化疗方案治疗有一定疗效,自体或异基因造血干细胞移植术有助于延长患者生存。