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.

Primary cutaneous anaplastic large cell lymphoma with overexpressed Ki-67 transitioning into systemic anaplastic large cell lymphoma:A case report

BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)differs from systemic anaplastic large cell lymphoma(sALCL)in cell biological behavior,clinical features,treatment,and outcome.PC-ALCL has been reported to rarely transition into sALCL,but the underlying mechanism is not clear.Here we report such a case with certain characteristics that shed light on this.CASE SUMMARY Herein,we report a 43-year-old male with symptoms of a skin nodule and histologically confirmed PC-ALCL with high expression of Ki-67.After three months of observation,two skin nodules re-appeared with muscle layer involvement and was histologically confirmed as sALCL.Seventeen months after receiving six cycles of CHOP regimen,the patient had pain in the chest and back,cough,shortness of breath,and night sweats.This was confirmed as relapse of sALCL by immunohistochemistry and several organs,such as the lung were involved as shown by positron emission tomography/computed tomography.After four cycles of DICE plus chidamide regimens followed by auto-hematopoietic stem cell transplantation(ASCT),complete remission(CR)duration was achieved for twelve months while the patient was on maintenance with chidamide(20 mg)pills.CONCLUSION This case had significantly high expression of Ki-67 when diagnosed as PC-ALCL initially and then transitioned into sALCL,which is rare.Auto-ASCT combined with demethylation drugs effectively maintained CR and prolonged progression free survival.

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.

Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography evaluation of subcutaneous panniculitis-like T cell lymphoma and treatment response

Subcutaneous panniculitis-like T cell lymphoma(SPTCL) is a very rare variant of non-Hodgkin's lymphoma. Currently, there is no standard imaging method for staging of SPTCL nor for assessment of treatment response. Here, we describe our use of fluorine-18 fluorodeoxyglucose(FDG) positron emission tomography/computed tomography(PET/CT) for staging and monitoring of treatment response in 3 cases of SPTCL. Primary staging by PET/CT showed that all 3 patients had multiple foci in the subcutaneous fat tissue, with SUVmax from 10.5 to 14.6. Involvement of intra-abdominal fat with high SUVmax was identified in 2 of the patients. Use of the triple drug regimen of gemcitabine, cisplatin and methylprednisolone(commonly known as "GEM-P") as first-line therapy or second-line therapy facilitated complete metabolic response for all 3 cases. FDG PET/CT provides valuable information for staging and monitoring of treatment response and can reveal occult involvement of the intraabdominal visceral fat. High FDG uptake on pre-treatment PET can identify patients with aggressive disease and help in selection of first-line therapy.

Low Dose Total Skin Electron Beam Radiation in Cutaneous T-Cell Lymphoma: Review

The treatment of advanced stage MF is especially challenging as single agent overall response rates are in the 35% range and chronic recurrence is the rule. The treatment of CTCL across all stages of disease is aimed at the goal of achieving and sustaining remission. Increasingly, low dose total skin electron beam therapy (TSEBT) is being utilized as a skin directed component in combination therapy for advanced stage CTCL. Researchers are seeking to better define the utility of low dose TSEBT as a method of debulking skin disease while simultaneously treating other disease compartments and in combination with sustained maintenance therapies of both the skin directed and systemic varieties. Data exists showing the efficacy of low dose TSEBT in early and advanced disease. There is also data documenting prolonged treatment responses with TSEBT plus adjuvant skin directed therapies such as PUVA and topical nitrogen mustard. Emerging data examining the role of low dose TSEBT in the prestem cell transplant preparation is also promising. This brief review summarizes the utility of low dose TSEBT in multiagent treatment regimens in CTCL.

NUCLEOLAR ORGANIZER REGIONS IN CUTANEOUS T CELL LYMPHOMAS

The present work studied the application of AgNOR count to differential diagnosis between cutaneous T cell lymphoma (CTCL) and cutaneous pseudolymphoma (CPL). Paraffin sections from 50 mycosis fungoides (22 MFI-Premycotic stage, 24 MF Ⅰ infiltrative stage and 4 MF Ⅲ - tumor stage), 2 nonepidermotropic cutaneous T cell lymphoma (NECTCL) and 9 CPL were investigated. In each case, 200 cells randomly selected were examined using a × 100 oil immersion lens. The mean number, standard deviation and standard error of the mean of AgNOR counts were as follows: MFⅠ 1.17±0.09, SEM = 0.01; MⅡ 1.17±0.01, SEM = 0.01; MF Ⅲ. 3.55±0.87, SEM = 0.43; NECTCL 4.5±0.28, SEM -0.199; CPL 1.17±0.1, SEM ± 0.03. The results revealed a highly significant difference between CTCL (MFⅢ+NECTCL) and CPL (t = 4.75, P<0.001), tumor stage (MF Ⅲ) and pretumor stage (MFI, MF Ⅱ) of mycosis fungoides (t = 4.75, P<0.001). Thus. AgNOR count is valuable in differential diagnosis.

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 Gamma Delta T Cell Lymphoma: A Clinicopathological Analysis

Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) is a very uncommon and extremely aggressive tumor, and is described in the newly re-vised World health organization for research and treatment of cancer classification of cutaneous lymphomas. A 43-year-old male patient presented with a 4 months history of cutaneous lesion over upper lip, without plaque and any constitutional symptom. Histopathological examination of skin biopsy revealed infiltration of atypical lymphocytes with hyperchromatic irregular nuclei. Immunophenotyping pattern of skin biopsy was compatible with PCGD-TLC. It is a highly aggressive tumor resistant to chemotherapy, immunotherapy, and radiation therapy. The GDTCL is characterized by a worse prognosis with a median survival of 15 months. Early diagnosis is essential and aggressive therapy is necessary.

Atypical Cutaneous Lymphoproliferative Disorder: A Fatal Mimic of Cutaneous T-Cell Lymphoma in a Patient with HIV Infection

Atypical cutaneous lymphoproliferative disorder (ACLD) is a rare condition that has been associated with HIV infection. Patients with ACLD present with diffuse, erythematous and pruritic skin lesions accompanied by generalized lymphadenopathy. The clinical characteristics of ACLD overlap most notably with several other conditions including Mycosis Fungoides/Sézary Syndrome (MF/SS), a cutaneous lymphoma of T-cell lineage. Unlike Mycosis Fungoides, the noxious infiltrates of ACLD are not monoclonal but polyclonal and consist of cytotoxic CD8+ T-cells instead of CD4+ T-cells or B-cells. Highly active antiretroviral therapy (HAART) has been reported to improve ACLD. We describe the case of a Caucasian man with longstanding HIV infection who presented with severe erythroderma. Skin and lymph node biopsies showed polyclonal CD8+ T-cell infiltrates. Gene rearrangement studies did not reveal an obvious clonal disorder. Hallmark peripheral blood findings consisting of a severe depletion of CD4+ T-lymphocytes and markedly elevated CD8+ cells provided an important diagnostic clue. Despite the purported benefits of HAART in ameliorating this disorder, erythroderma and extreme pruritus improved only after the patient began taking mycophenolate mofetil and hydroxyurea. Unfortunately, he succumbed to complications of methicillin-resistant Staphylococcus aureus septicemia. We alert readers to this rare HIV-associated condition which may mimic other benign and malignant skin conditions and briefly discuss diagnostic and therapeutic options.

Two Cases of Tuberculosis Manifesting as Cutaneous Solitary Mass in Patients with Adult T-Cell Leukemia/Lymphoma

Tuberculosis (TB) is a major public health problem worldwide and a large number of fatal cases are still reported. Immunocompetent individuals are naturally susceptible to TB, and immunocompromised patients have a greater risk of infection. Although patients with adult T-cell leukemia/lymphoma (ATL) are in an immunosuppressed condition, there is only one reported case of TB accompanied with ATL in the English- language literature in the field of dermatology. Here, we report two patients with chronic-type ATL infected with TB manifesting as cutaneous solitary masses. Case 1 was a 58-year-old woman diagnosed with lumbar abscess with pulmonary TB. Case 2 was an 84-year-old woman diagnosed with tuberculous lymphadenitis in the left cervical region. It is important to raise the differential diagnosis of TB and perform tissue culture for acid-fast bacilli as well as Interferon-Gamma release assay test when dermatologists encounter mass lesions in patients with ATL.

Late diagnosis and TCD8 immune response profile of cutaneous tuberculosis: A case report

Introduction: Cutaneous tuberculosis (CTB) is a rare form of extra-pulmonary tuberculosis that, when associated with late diagnosis, worsen the quality of life of the sick individuals. This report presents a case of late diagnosis of CTB. Unusual clinical manifestations retarded the correct tuberculosis diagnosis for more than a year. The immune response elicited by this type of tuberculosis as well as the factors that might contribute to the delay in diagnosis was evaluated and discussed. Methodology: Clinical evaluation and flow cytometric analyses of PBMC were realized for a case of CTB and a healthy individual as a control. Results: M. tuberculosis specific TCD8+ cell response was analyzed by flow cytometry and revealed positive cells for IL-17, IL-10, TGF-β and IDO. The CTB patient presented higher percentage of those cells when compared to a healthy donor. However, TCD8 cells positive for the important protective cytokine, IFN-γ was decreased in the CTB patient. Conclusion. The assessment of the M. tuberculosis specific TCD8+ immune response showed a regulatory/modulatory phenotype with a reduced IFN-γ response when compared to the healthy control that could have contributed to the CTB infection.

Clinicopathology, immunophenotype, T cell receptor gene rearrangement, Epstein-Barr virus status andp53 gene mutation of cutaneous extranodal NK/T-cell lymphoma, nasal-type

Background Extranodal natural killer/T-cell （NK/T cell） lymphoma, nasal-type, is a rare lymphoma. Skin is the second most common site of involvement after the nasal cavity/nasalpharynx. The aim of this study was to investigate the clinicopathologic features, immunophenotype, T cell receptor （TCR） gene rearrangement, the association with Epstein-Barr virus （EBV） infection and p53 gene mutations of the lymphoma. Methods The clinicopathologic analysis, immunohistochemistry, in situ hybridization for EBERI/2, TCR gene rearrangement by polymerase chain reaction （PCR）, mutations of p53 gene analyzed by PCR and sequence analysis were employed in this study. Results In the 19 cases, the tumor primarily involved the dermis and subcutaneous layer. Immunohistochemical staining showed that most of the cases expressed CD45RO, CD56, CD3E, TIA-1 and GrB. Three cases were positive for CD3 and two cases were positive for CD30. Monoclonal TCRy gene rearrangement was found in 7 of 18 cases. The positive rate of EBERI/2 was 100%. No p53 gene mutation was detected on the exon 4-9 in the 18 cases. Fifteen cases showed Pro （proline）/Arg （arginine） single nucleotide polymorphisms （SNPs） on the exon 4 at codon 72. The expression of p53 protein was 72% （13/18）immunohistochemically. Conclusions Cutaneous NK/T-cell lymphoma is a rare but highly aggressive lymphoma with poor prognosis. No p53 gene mutation was detected on the exon 4-9, and Pro/Arg SNPs on p53 codon 72 were detected in the cutaneous NK/T-cell lymphoma. The overexpression of p53 protein may not be the result of p53 gene mutation.

Cutaneous Manifestations and Treatment Advances of Adult T-Cell Leukemia/Lymphoma

Adult T-cell leukemia/lymphoma(ATLL)is an aggressive peripheral T-cell lymphoma caused by the human T lymphotropic virus type-1.The skin is affected in approximately half of ATLL patients,and skin lesions may be the first manifestation of the disease.The skin lesions of ATLL are polymorphous,and depend on the type of skin eruption,which makes it possible for doctors to predict the prognosis of the disease based on the characteristics of skin lesions.In this review article,we describe the clinical manifestations and histopathological patterns of skin lesions in ATLL,focus on its diagnostic and prognostic significance,and also summarize the advances in the treatment of ATLL.

Sezary syndrome： a rare form of cutaneous T-cell lymphoma

Sezary syndrome （SS） is an erythrodermic, aggressive, cutaneous T-cell lymphoma （CTCL） characterized by a malignant T-cell clone that localizes in the blood and skin, and its diagnosis is based on clinical, histological and biological features. Here we describe a typical case of SS.

EXPRESSION OF PROLIFERATING CELL NUCLEAR ANTIGEN IN CUTANEOUS MALIGNANT LYMPHOMAS AND LYMPHOID INFILTRATES

Paraffin-embedded tissue of skin biopsy specimens taken retrospectively from 24 patients with cutaneous malignant lymphomas (CML) and 8 patients with cutaneous lymphoid infiltrates (CLI) and other dennatoses were studied retrospectively with PCIO immunostaining. The results show a statistical significant difference among PCIO indices for cutaneous genuine histiocytic lymphoma (CGHL), cutaneous germinal center cell-derived lymphomas (CGCCL), cutaneous peripheral T-cell lymphomas (CPTL), non-mycosis fungoides (MF) and Sezary's syndrome (SS), and MF when compared with those for CLI. There is a linear relationship between PCIO index and square root of PCIO density, both of which seem to have a parallel relationship to the severity of malignancy in CML. The nuclear volume of the positive tumor cell or lymphocyte with PCIO immunostaining may be also useful in differentiating CML from CLI.

Genetic abnormalities assist in pathological diagnosis and EBVpositive cell density impact survival in Chinese angioimmunoblastic T-cell lymphoma patients

Objective:To explore the application of genetic abnormalities in the diagnosis of angioimmunoblastic T-cell lymphoma(AITL)and the reliable pathological prognostic factors.Methods:This study included 53 AITL cases,which were reviewed for morphological patterns,immunophenotypes,presence of Hodgkin and Reed-Sternberg(HRS)-like cells,and co-occurrence of B cell proliferation.The Epstein-Barr virus(EBV)-positive cells in tissues were counted,and cases were classified into“EBV encoded RNA(EBER)high-density”group if>50/HPF.Targeted exome sequencing was performed.Results:Mutation data can assist AITL diagnosis:1)with considerable HRS-like cells(20 cases):RHOA mutated in 14 cases(IDH2 co-mutated in 3 cases,4 cases with rare RHOA mutation),TET2 was mutated in 5 cases(1 case comutated with DNMT3A),and DNMT3A mutated in 1 case;2)accompanied with B cell lymphoma(7 cases):RHOA mutated in 4 cases(1 case had IDH2 mutation),TET2 mutated in 2 cases and DNMT3A mutated in 1 case;3)mimic peripheral T cell lymphoma,not otherwise specified(5 cases):RHOA mutated in 2 cases(IDH2 co-mutated in 1 case),TET2 mutated in 3 cases,and DNMT3A mutated in 1 case;4)pattern 1(1 case),RHOA and TET2 co-mutated.Besides RHOAG17V(30/35),rare variant included RHOAK18N,RHOAR68H,RHOAC83Y,RHOAD120G and RHOAG17del,IDH2R172 co-mutated with IDH2M397V in one case.There were recurrent mutations of FAT3,PCLO and PIEZO1 and genes of epigenetic remodeling,T-cell activation,APC and PI3K/AKT pathway.EBER high-density independently indicated adverse overall survival and progression-free survival(P=0.046 and P=0.008,KaplanMeier/log-rank).Conclusions:Over half AITL cases might be confused in diagnosis for certain conditions without mutation data.Targeted exome sequencing with a comprehensive panel is crucial to detect both hot-spot and rare mutation variants for RHOA and IDH2 and other recurrent mutated genes in addition to TET2 and DNMT3A.EBER highdensity independently indicated adverse survival.

鼻型结外NK/T细胞淋巴瘤误诊为鼻窦炎眶周蜂窝织炎1例

1临床资料患者,男,34岁,因“鼻堵脓涕1月余伴右眼红肿2周”于门诊就诊。患者1个月前在劳累饮酒后出现双侧持续性鼻堵、黄脓涕,伴双侧额部头痛、右眼眶重度胀痛,需口服止痛药缓解疼痛。2周前右眼疼痛加重,伴视物稍模糊,无复视,伴发热,体温最高38.2℃。门诊初步疑诊“急性鼻窦炎眶周蜂窝织炎”收治入院。发病以来患者神清,精神可,食欲尚可,近6个月体重下降约30斤,夜间睡眠差,伴乏力。

鼻型NK/T细胞淋巴瘤与慢性鼻窦炎的鉴别诊断要点

目的对比鼻型自然杀伤细胞/T细胞淋巴瘤(natural killer/T-cell lymphoma,NKTL)及慢性鼻窦炎(CRS)的临床特点,分析鉴别诊断要点。方法收集和对比29例鼻型NKTL及54例CRS病例资料,包括病史、临床表现、影像学资料。结果NKTL组症状出现时间较短(P<0.001),更常见涕中带血症状(P=0.002),鼻甲及鼻中隔黏膜弥漫肿胀(P<0.001)和鼻腔内结构破坏体征(P=0.024),MRI更多见下鼻甲(P=0.034)、鼻中隔(P<0.001)、鼻咽部(P=0.002)及颅底(P=0.024)受累,“铸形样”改变或鼻外浸润。CRS组更多见嗅觉减退症状(P<0.001),中鼻道肿胀及鼻腔内肿物(P均<0.001),CT更常见鼻窦骨质增生(P=0.002)。结论鼻型NKTL与CRS比较,症状持续较短、常见鼻腔弥漫肿胀伴鼻腔内外结构破坏,MRI多见下鼻甲、鼻中隔、鼻咽部和颅底受累,“铸形样”改变和“跳跃式”浸润。

淋巴结滤泡辅助T细胞淋巴瘤伴B细胞克隆性增生10例临床病理特征分析

目的探讨淋巴结滤泡辅助T细胞淋巴瘤血管免疫母细胞型(nodal follicular helper T cell lymphoma,angioimmunoblastic-type,nTFHL-AI)伴B细胞克隆性增生的临床病理学特征、免疫表型、分子特征、治疗及预后。方法收集10例nTFHL-AI伴B细胞克隆性增生患者的临床病理资料,分别行HE、免疫组化和基因重排检测,并复习相关文献。结果10例患者中男性5例,女性5例,中位年龄73岁。临床表现以全身淋巴结肿大、脾肿大及B症状为主;Ann Arbor分期:Ⅳ期8例,Ⅰ+Ⅱ期2例。实验室检查以β2微球蛋白和乳酸脱氢酶(LDH)升高,血红蛋白、红细胞和血小板减少为主,血浆EBV核酸定量阳性8例。镜下均可见典型结节状聚集或散在的透明细胞、分枝状高内皮血管及增生杂乱“风吹”状的滤泡树突网。肿瘤细胞背景中嗜酸性粒细胞浸润0~5个/HPF 7例,5~10个/HPF 2例,>50个/HPF 1例。浆细胞含量≤5%6例,浆细胞含量10%和20%各1例,浆细胞含量较多(30%)2例。组织细胞明显增生7例。见RS样大细胞1例。背景中含大量B细胞5例,含少量B细胞5例。10例患者均表达T细胞标志物,滤泡辅助性T细胞标志物CD10、BCL6、CXCL13和PD-1同时阳性6例,BCL6、CXCL13和PD-1同时阳性10例。EBER原位杂交阳性8例。10例患者均检测到TCR基因重排和IG基因重排阳性。所有患者诊断后均行化疗,其中3例疾病进展死亡。结论伴B细胞克隆性增生nTFHL-AI中,B细胞的克隆性增生与EBV是否感染及感染细胞的数量无关,同时浆细胞增生提示预后不良。