BACKGROUND:Primary cutaneous follicular helper T-cell lymphoma (pcTFH-L) is a rare, poorly characterized entity. We present six new cases to elucidate its clinicopathological features. METHODS:We retrospectively analyzed the clinical, histomorphological, and immunohistochemical features (TFH markers: PD-1, CXCL13, BCL-6, CD10, ICOS), EBV status, and T-cell receptor clonality of six pcTFH-L cases, comparing findings with 48 previously reported cases. RESULTS:The cohort (five males, one female; median age 66.5 years, range 42-75 years) presented with localized skin lesions without B symptoms or lymphadenopathy. All cases showed dermal/subcutaneous infiltration by atypical lymphocytes without epidermotropism (nodular pattern in 4 cases, diffuse pattern in 2 cases). Prominent high endothelial venules (HEVs) were exhibited in 4/6 cases. Neoplastic cells in all cases expressed CD4 and at least three TFH markers, with PD-1 (6/6), ICOS (6/6), and CXCL13 (6/6) being the most sensitive. CD21-positive follicular dendritic cell (FDC) networks were observed in 4 of 6 cases. Epstein-Barr virus-encoded RNA (EBER) was positive in 4 of 6 cases, and clonal T-cell receptor (TCR) gene rearrangements were detected in all 6 cases tested. Treatments varied from none to combination chemotherapy, with outcomes ranging from stable disease to complete remission; one patient died of heart failure, and another died at 20 months despite treatment. CONCLUSIONS:This case series reinforces pcTFH-L as a distinct cutaneous lymphoma with a characteristic immunophenotype and microenvironment. The consistent expression of multiple TFH markers, particularly PD-1, ICOS, and CXCL13, is essential for accurate diagnosis and differentiation from other cutaneous T-cell lymphoproliferative disorders. The clinical course appears heterogeneous, with some cases showing indolent behavior while others may have more aggressive outcomes.

BACKGROUND:Primary colonic Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+ iFDCS) is a rare, poorly characterized neoplasm. This study aims to delineate its clinicopathological features and thereby facilitate accurate diagnosis. METHODS:This study retrospectively analyzed 14 cases of primary colonic EBV+iFDCS and conducted a systematic review of 18 additional cases from the literature. RESULTS:The median age was 51 years (range, 35-68 years), with 10 females and 4 males. All cases were confined to the colon, with the transverse colon as the most common site (50%, 7/14), followed by the descending colon (28.6%, 4/14), sigmoid colon (14.3%, 2/14), and ascending colon (7.1%, 1/14). The vast majority of cases (85.7%, 12/14) were asymptomatic and were incidentally detected during routine physical examinations. Endoscopically, the tumors presented as either pedunculated polyps (9 cases) or sessile polypoid lesions (5 cases) with surface congestion. Their histology was characterized by mucosal erosion, prominent superficial perpendicular vessels, and secondary lymphoid follicular (SLF)hyperplasia. Neoplastic spindle cells were arranged in scattered and fascicular patterns within a background of plasma cells and small lymphocytes. Morphological subtypes comprised classic/lymphoma-like mixed type (64.3%, 9/14) and lymphoma-like type (35.7%, 5/14). Tumor cells expressed multiple FDC markers (CD21, CD23, CD35), co-expressed SMA (100%, 13/13) and CD56 (100%, 12/12), while SSTR2 was typically negative or only weakly expressed. The microenvironment was characterized by a B-cell predominance. EBV-encoded RNA was detected in perivascular and interfollicular tumor cells, with positivity also in germinal center FDCs. Clonality assays were negative in all but two cases (one case each with TCR rearrangement and IG rearrangement). All patients were underwent endoscopic submucosal resection, with no recurrence or major complications. CONCLUSION:Primary colonic EBV+iFDCS is a distinct, low-grade malignant neoplasm that typically presents as polyp or polypoid lesion. Its hallmark histologic features include a characteristic arrangement of vessels and SLFs in a prominent lymphocytic background. The tumor exhibits a dual immunophenotype, expressing markers of both FDCs and follicular reticular cells. Because the disease follows an indolent clinical course with a favorable prognosis, accurate diagnosis is essential to avoid overtreatment.

The differential diagnosis between adult systemic EBV-positive T-cell lymphoproliferative disorders (EBV+ T-LPD) and angioimmunoblastic T-cell lymphoma (AITL) with multiple EBV infections is difficult, and distinguishing between the two has become a diagnostic challenge for pathologists. Given that the clinical treatment plans are different, an accurate diagnosis is a prerequisite to ensure effective treatment, therefore, it is extremely necessary and meaningful to find effective pathological indicators for distinguishing between two diseases. We present a retrospective study comparing 7 cases of adult EBV+ T-LPD and 16 cases of AITL with multiple EBV infections diagnosed at our institution from 2017 to 2022. Differences in immunophenotype, type of EBV-infected cells, clonality and gene mutations between the two groups of cases were compared by immunohistochemical staining, double-label staining, TCR gene rearrangement and next-generation sequencing analysis. 7 cases of adult EBV+ T-LPD: all cases had no more than 1 T follicular helper (THF) marker was expressed, and there were significantly more EBER+/CD3 + cells than EBER+/CD20 + cells; 5 cases had mutation detection results, in which only 1 had the characteristic KMT2D mutation, 2 had TET2 mutations, and no common mutations such as DDX3X were detected.16 cases of AITL with multiple EBV infections: all cases were found to express at least 2 TFH markers, with 87

ABSTRACT:B-cell maturation antigen (BCMA) chimeric antigen receptor (CAR) T-cell therapy has demonstrated promising therapeutic efficacy in relapsed or refractory (R/R) multiple myeloma. However, distinct CAR T-cell constructs exhibit varying therapeutic outcomes. As the antigen-recognition domain, nanobodies offer a small, stable, single-domain structure with enhanced affinity and specificity compared with conventional single-chain variable fragments. We explored the use of nanobody-based BCMA(S103) CAR T-cell therapy for R/R plasma cell myeloma. The CAR construct incorporates dual-nanobody variable domain of the heavy chain of heavy chain antibody (VHHs) targeting BCMA. A cohort of 27 patients was treated with S103 CAR T-cell therapy, which included 4 patients of plasma cell leukemia, and 1 patient of anaplastic plasma cell myeloma. Eleven patients had multiple extramedullary lesions, and 11 patients exhibited high-risk genetic abnormalities, including 4 with TP53 mutations. One month after CAR T-cell infusion, the overall response rate (ORR) was 96.3% (26/27), with a complete response (CR) + very good partial response (VGPR) rate of 59.2% (16/27). At the 3-month follow-up, the ORR increased to 100% (27/27), with a CR + VGPR rate of 81.5% (22/27). The median duration of remission was 11 months (range, 2-36 months). The 1-year overall survival rate was 61.1%, and progression-free survival was 57.2%. In conclusion, BCMA CAR T-cell therapy, utilizing dual-nanobody VHHs targeting BCMA, demonstrates a high ORR and manageable safety profile in treating patients with R/R plasmacytic myeloma, including those with high-risk features such as extramedullary lesions, high-risk cytogenetic abnormalities, plasma cell leukemia, or anaplastic plasmacytoma. This trial was registered at www.ClinicalTrials.gov as #NCT04447573.

Benign natural killer cell enteropathy (NKCE) was first identified in the gastrointestinal (GI) tract. Notably, instances of NKCE have previously been observed at various sites other than the GI tract, including the gallbladder, lymph nodes, esophagus, and female genital tract. Typical NKCE manifests as an NK-cell immunohistological phenotype, with or without TCR rearrangement, and is characterized by the absence of Epstein-Barr virus (EBV) infection and protracted clinical progression. The misdiagnosis of NKT-cell lymphoma has resulted in some patients receiving chemotherapy, while in other instances, the patients' conditions resolved without treatment and showed no evidence of disease recurrence or progression during follow-up examinations. In this paper, we describe a unique case of EBV-negative NKCE occurring in the oral cavity, the first time such a case has been documented. The tumor completely resolved after an excisional biopsy, and subsequent follow-up did not reveal any signs of disease recurrence.

INTRODUCTION:Investigation of bone marrow (BM) trephine biopsies and/or clot sections by CD34 immunohistochemistry (IHC) testing has been used by pathologists for several decades, and its clinical value has been well established with QBEND10 being the most frequently used primary antibody (Ab) clone. However, most other parameters related to the IHC protocol as well as the readout vary widely between clinical laboratories and in the published literature. The ICSH Working Group having reviewed the published evidence has established guidelines that will help to harmonize performance and reporting of CD34 IHC on BM biopsies. METHODS:The methodology is based on the published "guidelines for guidelines" (GRADE, AGREE) with modifications to the specific laboratory medicine environment and to specific key questions. Review of the published literature resulted in 49 articles relevant to clinical applications of the BM CD34 IHC. Five key questions were addressed including testing indications, technical performance, readout methodology, terminology, and reporting. RESULTS:A total of 23 guidelines were grouped according to the key questions. CONCLUSION:BM CD34 IHC testing is complex with both the protocol and the pathologist's readout requiring validation to ensure reported results are reproducible and accurate. The readout/interpretation must be adapted to a specific purpose of the assay (clinicopathological question) and the type and overall sample quality. Standardized terminology and reporting are essential if CD34 IHC assay is being used for clinical diagnostics.

Objective: To investigate the clinical and pathologic features and diagnosis of follicular lymphoma (FL) with a predominantly diffuse growth pattern (DFL) with 1p36 deletion. Methods: Eight cases of DFL with 1p36 deletion diagnosed at Department of Pathology, Beijing Friendship Hospital, Capital Medical University (n=5) and the Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital (n=3) from January 2017 to January 2023 were included. Their clinicopathologic features and follow-up data were analyzed. Immunohistochemistry and fluorescence in situ hybridization (FISH) were performed. Results: There were five males and three females, with a median age of 67 years, and inguinal lymphadenopathy was found as the main symptom. Histologically, similar morphologic features were sheared among all cases, with effaced nodal structure and characterized by proliferation of centrocytes in a diffuse pattern, with or without follicular components. The germinal center-related markers such as CD10 and/or bcl-6 were expressed in the tumor cells, and 1p36 deletion but not bcl-2 translocation was appreciable in these cases. Conclusions: DFL with 1p36 deletion is a rare subtype of FL, with some overlaps with other types of FL or indolent B-cell lymphomas in their pathologic features. An accurate diagnosis requires comprehensive considerations based on their clinical, pathologic, immunohistochemical, and molecular features.

Objective: To investigate the clinicopathological characteristics of rashes in monkeypox patients through a series of skin biopsies, and examine their pathological features and the most effective tests. Methods: Patients with monkeypox virus infection admitted to Beijing Ditan Hospital from June to August 2023 were identified. Among them, 24 patients underwent skin biopsies for clinical pathological study that were included in this study. Clinical information, rash pictures, and nucleic acid test results were analyzed using histopathology, immunohistochemistry, RNAscope® hybridization and electron microscopy. Results: All 24 patients were male, including 14 patients with concurrent human immunodeficiency virus infection. Their average age was (32.3±5.4) years. The nucleic acid test confirmed monkeypox virus infection. The clinical feature of monkeypox rashes was solitary rather than clustered distribution, with rashes occurring in similar phase, distinguishing it from herpesvirus. The rashes in these patients were mostly scattered, with an average of (13.0±11.8) rashes, and most commonly present in the perineum, face, limbs, and trunk. The three main pathological features of these rashes were ballooning degeneration of the epidermal spinous cell layer, the characteristic intra-cytoplasmic Guarnieri's bodies and significant infiltration of inflammatory cells in whole dermal layer. Immunohistochemistry, RNAscope® hybridization, and electron microscopy can all effectively detect the monkeypox virus. Electron microscopy showed viral replication in various types of skin cells. Conclusions: The study describes the pathological features of monkeypox virus rashes. Pathological examination of skin biopsy samples is helpful to diagnose these rashes. The study suggests that the monkeypox virus has a unique epitheliotropic affinity and can infect various types of cells in the skin.

Objective: To study the clinicopathological features of Sjogren's syndrome (SS) with liver injury and to improve the understanding of this disease. Methods: Forty-nine patients with SS complicated with liver injury were collected from Beijing Ditan Hospital, Capital Medical University from October 2008 to January 2022. All patients underwent ultrasound-guided liver biopsy, and all specimens were stained with HE. The histopathologic characteristics were observed and the pathologic indexes were graded. Immunohistochemical stains for CK7, CK19, CD38, MUM1 and CD10 were performed by EnVision method; and special histochemical stains for reticulin, Masson's trichrome, Rhodanine, Prussian blue, periodic acid Schiff (PAS) and D-PAS stains were conducted. Results: The age of patients ranged from 31 to 66 years, including 3 males and 46 females. SS combined with drug-induced liver injury was the most common (22 cases, 44.9%), followed by autoimmune liver disease (13 cases, 26.5%, including primary biliary cholangitis in eight cases, autoimmune hepatitis in 3 cases, and PBC-AIH overlap syndrome in 2 cases), non-alcoholic fatty liver disease (NAFLD, 9 cases, 18.4%) and other lesions (5 cases, 10.2%; including 3 cases of nonspecific liver inflammation, 1 case of liver amyloidosis, and 1 case of porto-sinusoidal vascular disease). Among them, 28 cases (57.1%) were associated with obvious interlobular bile duct injury, mainly in SS combined with PBC group and drug-induced liver injury group. Twenty-three cases (46.9%) were associated with hepatocyte steatosis of varying degrees. In SS with autoimmune liver disease group, ISHAK score, degree of fibrosis bile duct injury, bile duct remodeling, lymphocyte infiltration of portal area, and plasma cell infiltration, MUM1 and CD38 expression; serum ALP and GGT, IgM; elevated globulin; positive AMA, proportion of AMA-M2 positive and IgM positive were all significantly higher than those in other groups(all P<0.05). Serum ALT, direct bilirubin and SSA positive ratio in SS combined with drug liver group were significantly higher than those in other groups(all P<0.05). The serum total cholesterol level in SS combined with PBC group (P=0.006) and NALFD group (P=0.011) were significantly higher than those in other groups (P<0.05). Conclusions: The pathologic manifestations of SS patients with liver injury are varied. The inflammatory lesions of SS patients with autoimmune liver disease are the most serious, and the inflammatory lesions of SS patients with non-alcoholic fatty liver disease and non-specific inflammation are mild. Comprehensive analysis of liver histopathologic changes and laboratory findings is helpful for the diagnosis of SS complicated with different types of liver injury.

Objective: To investigate the clinicopathological features and differential diagnosis of primary mucosal CD30-positive T-cell lymphoproliferative disorders (pmCD30+TLPD). Methods: Eight cases of pmCD30+TLPD diagnosed from 2013 to 2023 at the Department of Pathology, Beijing Friendship Hospital Affiliated to Capital Medical University and Beijing Ludaopei Hospital were retrospectively collected. The immunophenotype, EBV infection status and T-cell receptor (TCR) clonability of tumor cells were examined. The clinicopathological features were analyzed and related literatures were reviewed. Results: There were 5 females and 3 males, aged 28 to 73 years, without B symptoms, lack of trauma and autoimmune diseases. Seven cases occurred in oral mucosa and one in anal canal mucosa. Submucosal nodules with ulcerations were presented in all cases except one, which only submucosal nodule. Morphologically, there was different distribution of allotypic lymphocytes in inflammatory background. Four cases showed "kidney-shaped", "embryonic" and "horseshoe-shaped" cells, and one case resembled Hodgkin and Reed/Sternberg (HRS) cells. Allotypic lymphocytes expressed CD3 (7/8), CD4+/CD8-(7/8) and CD4-/CD8-(1/8). CD30 was uniformly strongly positive while ALK and CD56 were negative. In situ hybridization of EBER was negative in five cases (5/5). Clonal TCR gene rearrangement was positive in two cases. Four patients did not receive radiotherapy or chemotherapy. All the seven patients survived without disease except one died due to concurrent leukopenia. Conclusions: pmCD30+TLPD had a broad morphological spectrum and could be easily confused with primary cutaneous CD30+TLPD and systemic ALK-negative anaplastic large cell lymphoma involving mucosa, which may lead to misdiagnosis. Although the majority of the cases had a favorable prognosis, a few cases relapsed or progressed to lymphoma.

Epstein-Barr virus (EBV) typically infects B cells in infectious mononucleosis (IM), but a rare case shows EBV infection in T cells. Seven cases of lymphoproliferative disorder caused by EBV-positive cytotoxic T/natural killer (NK) cell proliferation in the lymph nodes, termed IM with transient EBV infection of T and NK cells (EBV + T/NK cells in IM), are reported here. The purpose of the study is to describe clinicopathological features of EBV + T/natural killer (NK) cells in IM of the lymph node. We retrospectively analysed seven cases of Chinese children and young people adults with EBV + T/NK cells in IM. We used morphological observation, immunohistochemical staining, EB virus in situ hybridisation detection, and analysis of T-cell receptor gene rearrangement. The patients were healthy prior to illness, experiencing sudden onset occurring in all the patients, with high fever as the first symptom, followed by lymphadenopathy and hepatosplenomegaly. Diagnosis occurred < 1.5 months of symptom onset. Most lymphocytes in lesions expressed CD3 and Granzyme B or TIA-1 and lacked CD5. CD56 was expressed in numerous cells in 5 of the 7 cases. EBV-encoded RNA (EBER) was detected in medium-to-large-sized cells (50–100 cells per cell/high-power field). T-cell receptor (TCR) gene rearrangement was seen in six cases, with monoclonal rearrangement in four cases. Treatment was conservative treatment but not chemotherapy. Four received anti-HLH therapy and others anti-inflammatory treatment. All patients survived with relapse after long-term clinical observation and follow-up. EBV + T/NK cells in IM can elicit malignant features that mimic T/NK-cell lymphoma pathologically and benign features mimicking IM clinically. These findings indicate that EBV + T/NK cells in IM could serve as valuable diagnosis. Additional clinical information, including age of onset (children and young people), nature of onset (sudden), disease course (short), symptoms (systemic), EBV infection status (acute), and lymph node involvement, is crucial for accurate diagnosis and prognostic evaluation.

Objective: To investigate the clinical, pathological and immunophenotypic features, molecular biology and prognosis of fibrin-associated large B-cell lymphoma (LBCL-FA) in various sites. Methods: Six cases of LBCL-FA diagnosed from April 2016 to November 2021 at the Beijing Friendship Hospital, Capital Medical University, Beijing, China and the First Affiliated Hospital, Wenzhou Medical University, Wenzhou, China were collected. The cases were divided into atrial myxoma and cyst-related groups. Clinical characteristics, pathological morphology, immunophenotype, Epstein Barr virus infection status, B-cell gene rearrangement and fluorescence in situ hybridization of MYC, bcl-2, bcl-6 were summarized. Results: The patients' mean age was 60 years. All of them were male. Three cases occurred in atrial myxoma background, while the others were in cyst-related background, including adrenal gland, abdominal cavity and subdura. All cases showed tumor cells located in pink fibrin clot. However, three cyst-related cases showed the cyst wall with obviously fibrosis and inflammatory cells. All cases tested were non germinal center B cell origin, positive for PD-L1, EBER and EBNA2, and were negative for MYC, bcl-2 and bcl-6 rearrangements, except one case with MYC, bcl-2 and bcl-6 amplification. All of the 5 cases showed monoclonal rearrangement of the Ig gene using PCR based analysis. The patients had detailed follow-ups of 9-120 months, were treated surgically without radiotherapy or chemotherapy, and had long-term disease-free survivals. Conclusions: LBCL-FA is a group of rare diseases occurring in various sites, with predilection in the context of atrial myxoma and cyst-related lesions. Cyst-related lesions with obvious chronic inflammatory background show more scarcity of lymphoid cells and obvious degeneration, which are easy to be missed or misdiagnosed. LBCL-FA overall has a good prognosis with the potential for cure by surgery alone and postoperative chemotherapy may not be necessary.

Objective: To investigate the clinicopathologic features of progressively transformed germinal center-like follicular T-cell lymphoma (PTGC-like FTCL). Methods: The clinicopathologic data of 14 PTGC-like FTCL cases that were diagnosed at the Beijing Friendship Hospital Affiliated to the Capital Medical University from January 2017 to January 2022 were retrospectively collected. Clinicopathological features, immunophenotype, and Epstein-Barr virus (EBV) infection status were analyzed in these cases. Polymerase chain reaction (PCR) was performed to detect the clonal gene rearrangements of T cell receptor (TCR) and the immunoglobulin (Ig) in 10 and 8 cases, respectively. Results: The male to female ratio was 5∶2. The median age was 61 years (range 32-70 years). All patients had lymphadenopathy at the time of diagnosis. By using the Ann Arbor system staging, seven cases were classified as stage Ⅰ-Ⅱ, and seven cases as stage Ⅲ-Ⅳ. Seven cases had B symptoms, four cases had splenomegaly, and two cases had skin rash and pruritus. Previously, three cases were diagnosed as classic Hodgkin's lymphoma, three cases as small B-cell lymphoma, two cases as atypical lymphoid hyperplasia unable to exclude angioimmunoblastic T-cell lymphoma (AITL), one case as EBV-associated lymphoproliferative disorder, and one case as peripheral T-cell lymphoma (PTCL) associated with the proliferation of B cells. All the 14 cases showed that the large nodules were composed of mature CD20+, IgD+B lymphocytes admixed with small aggregates of neoplastic cells with pale to clear cytoplasm. Moreover, hyperplastic germinal centers (GCs) and Hodgkin/Reed-Sternberg-like (HRS-like) cells were seen within these nodules in two and five cases, respectively. The neoplastic cells expressed CD3 (14/14), CD4 (14/14), PD1 (14/14), ICOS (14/14), CD10 (9/14), bcl-6 (12/14), CXCL13 (10/14), and CD30 (10/14). The HRS-like cells in five cases expressed CD20 (2/5), PAX5 (5/5), CD30 (5/5), CD15 (2/5), LCA (0/5), OCT2 (5/5) and BOB1 (2/5). Moreover, neoplastic T cells formed rosettes around HRS-like cells. EBV-encoded RNA (EBER) in situ hybridization showed scattered, small, positive bystander B lymphocytes in 8/14 cases, including 3/5 cases containing HRS-like cells. All tested cases (including five with HRS-like cells) showed monoclonal TCR gene rearrangement and polyclonal Ig gene rearrangement. Conclusions: PTGC-like FTCL is a rare tumor originated from T-follicular helper cells. It could be distinguished from angioimmunoblastic T-cell lymphoma by the formation of follicular structure, and lack of follicular dendritic cell proliferation outside the follicles and the polymorphous inflammatory background. In addition, it should be differentiated from lymphocyte-rich classical Hodgkin's lymphoma and low-grade B cell lymphoma.

目的： 探讨EB病毒阳性皮肤黏膜溃疡（EBVMCU）的临床病理学特征和鉴别诊断。 方法： 收集首都医科大学附属北京友谊医院病理科2016—2022年会诊的6例EBVMCU，行HE染色、免疫组织化学染色及原位杂交检测，并复习相关文献。 结果： 6例EBVMCU患者中，男性5例，女性1例，年龄43~79岁。均无B症状。5例均为表浅、界限清楚的溃疡，异型大细胞散在或簇状分布于多形性炎性背景中，部分呈HRS样；仅1例大细胞呈片状分布。异型大细胞均表达CD20、CD30和MUM1，Ki-67阳性指数均>50%。CD10均阴性。EB病毒原位杂交均阳性。3例完全缓解，1例部分缓解，1例失访，1例进展为EB病毒阳性弥漫性大B细胞淋巴瘤。 结论： EBVMCU形态学谱系较宽，容易与其他EB病毒阳性B细胞淋巴组织增殖性疾病或淋巴瘤混淆。本病需特别强调整合临床、病理、影像学及实验室检查信息综合诊断。.

目的 探讨以腹腔淋巴结肿大为首发表现的淋巴瘤的病理类型及构成特点.方法 回顾性分析216例淋巴瘤的临床资料、病理组织学类型及免疫组织化学染色结果,按《WHO(2017)造血与淋巴组织肿瘤分类》标准进行病理诊断及分类.结果 216例淋巴瘤中,男性139例,女性77例,男女比例为1.8:1.年龄2～77岁,平均年龄43.5岁,中位年龄41岁.患者均以发现腹腔淋巴结肿大为首发表现就诊.其中121例(56.0％)无伴随症状,在体检时偶然发现;95例(44.0％)患者伴有不同程度的发热(29例,13.4％)、腹胀(68例,31.5％)、盗汗(41例,19.0％)及体重减轻(39例,18.1％).淋巴结直径1～13 cm,平均6.4cm.非霍奇金淋巴瘤(NHL)204例(94.4％),霍奇金淋巴瘤(HL)12例(5.6％).NHL中B细胞来源185例(85.6％),T细胞或NK细胞来源19例(8.8％).NHL-B细胞来源中,构成比居前3位的分别为滤泡性淋巴瘤(FL)92例(42.6％),弥漫大B细胞淋巴瘤-非特殊类型(DLBCL-NOS)54例(25.0％)、Burkitt淋巴瘤23例(10.6％).结论 以腹腔淋巴结肿大为首发表现的淋巴瘤以男性多见,滤泡性淋巴瘤是最常见的类型.无论哪种类型,临床症状均不典型,需引起注意,避免漏诊.

Objective: To investigate the clinicopathological features and prognosis of cytotoxic T-cell lymphoma (CTL). Methods: The clinicopathological data of 134 CTL patients in Beijing Friendship Hospital Affiliated to Capital Medical University, the 989 Hospital of PLA Joint Logistics Support force (formerly the 152 Hospital) and the Fourth Hospital of Hebei Medical University from 2008 to 2020 were retrospectively collected. Immunophenotype, Epstein-Barr virus infection status and T cell receptor (TCR) clonality of tumor cells were assessed, and clinicopathological features and prognosis of patients were analyzed. Results: Among the 134 CTL patients, the male to female ratio was 1.7∶1.0, the median age was 49.5 years (range 3-83 years), and 100 cases (74.6%) were under 60 years old. Forty-six point nine percent of the patients (53/113) had B symptoms. Most of the patients presented with systemic superficial lymphadenopathy. According to the Ann Arbor staging system, 36.8% (39/106) of the patients were in stage Ⅰ-Ⅱ, and 63.2% (67/106) in stage Ⅲ-Ⅳ. The rate of extranodal involvement was 51.6% (66/128). Spleen was involved in 24.2% (31/128) of the cases. Morphology showed diffuse growth of abnormal lymphocytes, infiltrating and destroying normal tissue structure. Immunohistochemical staining showed that tumor cells expressed T cell antigens (CD2, CD3, CD5, and CD7), and 72.0% (77/107) of them had decreased or lost expression of one or more antigens. According to the numbers of CD4 and CD8 expression in tumor cells, 70 cases (52.2%) were grouped into CD8+>CD4+group. The expression rates of TIA-1 and granzyme B were 99.2% (119/120) and 79.8% (95/119), respectively. CD20 abnormal expression rate was 27.6% (37/134) and CD56 was negative in all cases. The median Ki-67 proliferative index was 45.0% (range 5%-80%). In situ hybridization of small RNA encoded by Epstein-Barr virus was negative. Clonal TCR gene rearrangement analysis was performed on 49 cases and was positive in all cases. Ninety-one patients were followed up for a median of 36 months (range, 1 to 240 months), and 40 of the 91 patients (44.0%) died. The twenty-three patients were in complete remission (including 13 cases with localized single extranodal mass). The 3-year and 5-year overall survival rates were 53.5% and 49.4%, respectively. Univariate analysis showed that B symptom, spleen involvement, extranodal involvement, clinical stage, CD8+>CD4+phenotype, abnormal expression of CD20 and Ki-67 proliferation index (>60%) were associated with overall survival (P<0.05). The multivariate Cox regression analyses showed that spleen involvement and CD8+>CD4+ phenotype were independent prognostic factors for overall survival in CTL patients. Conclusions: CTL are more commonly found in adult males under 60 years old, often accompanied by B symptom, with a high proportion of extranodal involvement and more CD8 positive phenotypes. Spleen involvement and CD8+>CD4+phenotype are independent predictors of CTL overall survival. Some patients with localized extranodal CTL may have a good prognosis.

Objective: To investigate the clinicopathological features of angioimmunoblastic T-cell lymphoma pattern Ⅰ (AITL Pattern Ⅰ). Methods: The clinicopathological data of 11 AITL Pattern Ⅰ cases that were diagnosed at the Beijing Friendship Hospital Affiliated to Capital Medical University (10 cases) and Beijing Lu Daopei Hospital (1 cases) from January 2019 to October 2021 were retrospectively collected. Immunophenotype, Epstein-Barr virus infection status and T cell receptor (TCR) clonality of the tumor cells were tested, and clinicopathological features of cases were analyzed. Results: Among the 11 AITL Pattern Ⅰ cases, the male to female ratio was 1.2∶1.0. The median age was 59 years (range 47-78 years). Seven cases had B symptoms, while eleven cases presented with systemic lymphadenopathy. According to Ann Arbor system staging, two cases were classified as stage Ⅰ-Ⅱ, and 9 cases as stage Ⅲ-Ⅳ. Hepatosplenomegaly was present in two cases (2/11), three cases (3/11) had skin rash and pruritus, and two cases (2/11) had pleural effusion. Previously, 6 cases (6/11) were diagnosed as reactive hyperplasia, 1 case (1/11) as EBV-associated lymphoproliferative disorder, and 4 cases (4/11) as hyperplasia of lymphoid tissue, which was unable to exclude lymphoma. Histologically, all the 11 cases showed hyperplastic follicles in the paracortical regions with well-formed germinal centers. The hyperplastic follicles showed ill-defined borders and attenuated mantle zones in 7 cases. Mantle zones completely disappeared in 4 cases. The follicles were surrounded by a thin layer of atypical lymphocytes with bright or faintly stained cytoplasm. In 2 cases, the clear cells were located between the germinal centers and the thin residual mantle cell layers, showing a circular growth pattern. The cells were medium in size, with irregular karyotype, coarse chromatin and indistinct nucleoli. Immunohistochemically, CD21 staining showed that the meshworks of follicular dendritic cells(FDC)were mainly confined to the follicles. There was a subtle expansion of the meshworks of FDC in 4 cases with ill-defined borders. The atypical cells surrounding the follicles expressed CD3 (11/11), CD4 (11/11), PD-1 (11/11), CXCL13 (6/11), ICOS (10/11) and CD10 (7/11). PD-1 staining showed a strong perifollicular pattern, and a small number of positive cells were scattered around the high endothelial veins in the interfollicular region. CXCL13, ICOS and CD10 showed similar distribution patterns. EBV-encoded small RNA probe (EBER) in situ hybridization showed that EBER positive B lymphocytes were scattered in the interfollicular region (5-20/HPF) in all cases. T cell receptor gene rearrangement was monoclonal in all cases. Conclusions: Diagnosing AITL Pattern Ⅰ may be challenging and requires comprehensive analysis of clinical manifestations, histological morphology, immunophenotype and gene rearrangement results.

Extramedullary blast crisis (EBC) is a special kind of blast crisis of chronic myelogenous leukemia (CML). It is more likely to be misdiagnosed as lymphoma when EBC cells are of lymphoid cell lineage and lymphadenopathy is the only symptom before the final diagnosis. In this study, we presented a patient with an unusual presentation of CML transformation as a rapid growth of generalized lymphadenopathy that appeared 5 months after the initial diagnosis of CML. The patient underwent the left supraclavicular lymph node biopsy and repeat bone marrow aspiration. The revealed CD3+, terminal deoxynucleotidyl transferase (TdT)+, CD5+, CD23+, myeloperoxidase (MPO)-, CD20-, cyclin D1-, CD10-, which was consistent with the diagnosis of T-cell lymphoblastic lymphoma (T-LBL). Fluorescence in situ hybridization (FISH) verified the BCR-ABL rearrangement, and T-cell EBC of CML was finally diagnosed. Our report suggested that FISH was necessary to distinguish isolated lymphoid extramedullary blast crisis from secondary NHL in CML.

目的 探讨原发性椎管内硬膜外淋巴瘤(PSEL)患者的性别、年龄及病理类型构成特点.方法 回顾性分析2008-01-2019-12首都医科大学附属北京友谊医院病理科本院外检及会诊病例中诊断为PSEL的病例共49例,分析患者的性别、发病年龄等临床资料及病理类型.结果 49例PSEL病例中,男性30例,女性19例,男女比为1.58∶1;年龄范围1～76岁,平均年龄40岁,中位年龄40岁,发病年龄与组织学类型有很大的关联,弥漫性大B细胞淋巴瘤(DLBCL)、黏膜相关淋巴组织(MALT)边缘区B细胞淋巴瘤(MALT淋巴瘤)、浆细胞瘤、滤泡性淋巴瘤(FL)更多见于中老年人,而B淋巴母细胞性淋巴瘤、Burkitt淋巴瘤(BL)更多见于儿童及青少年.病理类型上,霍奇金淋巴瘤(HL)3例(6.1％),非霍奇金淋巴瘤(NHL)46例(93.9％).3例HL均为经典型霍奇金淋巴瘤(CHL),2例为结节硬化型,1例难以分出具体类型.46例NHL中,B细胞淋巴瘤占97.8％(45例),T/NK细胞淋巴瘤占2.2％(1例).B细胞淋巴瘤中前五位为:DLBCL 35.6％(16例),B淋巴母细胞性淋巴瘤22.2％(10例),MALT淋巴瘤11.1％(5例),浆细胞瘤8.9％(4例),BL 6.7％(3例),FL 6.7％(3例).1例T/NK细胞淋巴瘤为细胞毒性外周T细胞淋巴瘤.结论 PSEL中NHL最为多见,HL较少见.NHL中以B细胞淋巴瘤为主,T/NK细胞淋巴瘤较少见.各个类型淋巴瘤的性别及发病年龄也各有特点.

淋巴浆细胞淋巴瘤（LPL）是由小B淋巴细胞、浆细胞样淋巴细胞和浆细胞组成的肿瘤，可累及骨髓、淋巴结和脾脏等部位。骨髓穿刺活检多见，淋巴结为首次活检诊断极具挑战性。该文讨论肺癌术后多发淋巴结肿大病例，形态学上滤泡间区大量浆细胞及小淋巴细胞，免疫表型支持B细胞和浆细胞，分子检测B细胞克隆性重排和MYD88突变。LPL形态学及免疫表型与其他小B细胞淋巴瘤类似，给临床病理诊断带来困难，容易造成误诊或漏诊，需提高警惕。