8p11骨髓增殖综合征的临床病理学特征

本研究旨在提高对罕见的8p11骨髓增殖综合征(eight p11myeloproliferative syndrome,EMS)的临床病理特征、诊断与治疗的认识。应用骨髓细胞涂片、骨髓活检观察细胞形态学改变,流式细胞术检测骨髓细胞的免疫表型,细胞遗传学方法分析骨髓细胞核型,分子生物学检测bcr/abl融合基因。结果表明:EMS是一组具有独特临床和生物学特点的疾病,以bcr/abl阴性的骨髓增殖性肿瘤合并淋巴母细胞淋巴瘤(lymphoblastic lymphoma,LBL)为主要特征。骨髓细胞形态学提示粒系细胞高度增生、嗜酸粒细胞增多;免疫表型分析显示髓系抗原表达增高;细胞遗传学分析表明存在8p11易位;RT-PCR检测bcr/abl融合基因呈阴性。在分子学水平,患者染色体异常均累及8p11上的成纤维细胞生长因子受体-1(FGFR1),目前共发现11种与FGFR1重排相关的伙伴基因,其中最常见的是位于13q11-12上的ZNF-198。EMS预后不良,患者常在短期内进展为急性髓系白血病,常规化疗效果差,除异基因造血干细胞移植外无有效的治疗方法。结论:EMS是伴有FGFR1重排的骨髓和淋巴肿瘤,易误诊为T-LBL、不典型慢性粒细胞白血病(aCML)及慢性粒-单核细胞白血病(CMML),对该病应及时进行细胞遗传学及分子生物学检测,以避免误诊、误治。     

8p11骨髓增殖综合征的研究现状

8p11骨髓增殖综合征(8p11 myeloproliferative syndrome,EMS)是与定位于髓系和淋巴系细胞8号染色体短臂(8p11)的成纤维细胞生长因子受体1(FGFR1)基因易位相关的侵袭性肿瘤。EMS以外周血白细胞计数明显增高、骨髓中髓系增生、嗜酸细胞增多和淋巴母细胞淋巴瘤/白血病为特征。EMS短期内将进展为急性白血病。EMS预后差,目前只有异基因造血干细胞移植能有效控制该病。在分子水平,所有病例均涉及8p11的FGFR1基因易位,新融合蛋白通过诱导FGFR1基因二聚体化,组成性激活酪氨酸激酶活性,导致细胞增殖及恶性转化。迄今,已鉴定出的与FGFR1形成融合蛋白的伙伴基因有14例(包括13例易位与1例插入),现就其分子生物学特征、发病机制和治疗等的研究现状作一综述。     

异基因造血干细胞移植治疗伴RUNX1胚系突变家族性血小板疾病伴急性髓系白血病1例报告并文献复习北大核心CSCD

随着基因测序技术的进步, 家族性血小板疾病伴髓系恶性肿瘤(FPD/MM)的诊断率越来越高。2016年WHO在造血淋巴组织肿瘤疾病分类首次提出了胚系易感性髓系肿瘤的概念, 其中大多数为遗传易感性骨髓增生异常综合征(MDS)、MDS/骨髓增殖性肿瘤(MPN)、急性髓系白血病(AML), 极少部分转化为淋系肿瘤。我们应用异基因造血干细胞移植(allo-HSCT)治疗1例伴RUNX1胚系突变的FPD/AML患者, 报道如下并对相关文献进行复习。     

PICAML-MLLT10融合基因阳性急性髓系白血病4例报告并文献复习北大核心CSCD

10号染色体上的PICALM基因与11号染色体上MLLT10基因融合形成PICALM-MLLT10融合基因,是淋巴细胞系及髓系血液系统恶性肿瘤中较为罕见的融合基因,该融合基因参与诱导白血病的发生且与不良预后密切相关[1,2,3]。由于发生率低,既往报道少,异基因造血干细胞移植(allo-HSCT)对于该类患者的疗效更是未知。我们回顾性分析了2017年至2021年在苏州大学附属第一医院接受allo-HSCT治疗的4例PICALM-MLLT10融合基因阳性急性髓系白血病(AML)患者的临床和实验室资料并进行相关文献复习。     

新诊断慢性髓系白血病髓外T淋巴细胞急变患者的临床特点分析

目的:报道1例新诊断慢性髓性白血病(chronic myelogenous leukemia,CML)髓外T淋巴细胞急变的患者的临床特点,以提高对该疾病的认识。方法:回顾性分析该患者的临床特征、诊疗方法,并对相关文献进行复习。结果:结合血常规、骨髓学、染色体和融合基因检查结果,考虑该患者为CML,患者淋巴结病理提示为T淋巴母细胞淋巴瘤;荧光原位杂交检测发现淋巴结病变中存在BCR-ABL融合信号,最终诊断为CML髓外T淋巴细胞急变。给予达沙替尼(140 mg/d)联合化疗,患者一直未达完全缓解。结论:新诊断的CML急变期患者相对少见,以CML髓外T淋巴细胞急变为特点并伴有附加染色体11q23的患者更为罕见。CML髓外T淋巴细胞急变的患者预后差,异基因造血干细胞移植或许是唯一有希望治愈的治疗方法。     

伴U2AF1基因突变骨髓增生异常综合征患者临床特征及预后分析

骨髓增生异常综合征(MDS)是一组起源于造血干细胞的异质性髓系克隆性疾病,多种基因突变在MDS预后判断中有重要价值[1-3]。10%~21%的MDS患者可检测到U2AF1基因突变,U2AF1基因突变的患者进展为急性髓系白血病(AML)的风险增高[4-6],而这类患者对化疗或异基因造血干细胞移植(allo-HSCT)治疗反应的报道较少。本研究我们回顾性分析59例U2AF1基因突变MDS患者的临床特征,比较化疗及allo-HSCT的疗效,并分析影响移植患者预后的危险因素。     

荧光原位杂交技术在异基因造血干细胞移植监测中的作用

背景：异基因造血干细胞移植后,嵌合率下降与移植物被排斥及白血病复发密切相关,常规骨髓细胞学检查、染色体检查、流式细胞学检查等均不能敏感特异地监测嵌合率。目的：验证荧光原位杂交技术检测异基因造血干细胞移植后嵌合率的敏感性和特异性。方法：应用荧光原位杂交法检测异性同胞异基因造血干细胞移植后患者X、Y性染色体及慢性粒细胞白血病序列特异性bcr/abl融合基因,以骨髓细胞学检查为对照。结果与结论：3例异基因造血干细胞移植患者共复查骨髓细胞学检查及荧光原位杂交检查7次。4次骨髓细胞学检查及荧光原位杂交检查均完全相符,另外3次骨髓细胞学检查提示完全缓解,而荧光原位杂交检查能检测到少量异常信号,证实应用荧光原位杂交法较骨髓细胞学具有显著优越性,能动态敏感、特异地检测嵌合率。     

伴有FGFR1重排的白血病/淋巴瘤一例报告附文献复习

伴有FGFR1重排的白血病/淋巴瘤,即8p11骨髓增殖综合征(EMS),是一种较罕见的血液系统恶性肿瘤,以髓系增生异常、嗜酸粒细胞增多、常伴发淋巴母细胞淋巴瘤(lymphoblast lymphoma,LBL)为其主要特征.染色体易位均涉及8号染色体短臂(8p11),即成纤维细胞生长因子受体-1(fibroblast growth factor receptor-1,FGFR1)基因位点,形成融合基因.其产物构成性激活酪氨酸激酶活性,通过多条信号传导通路影响细胞的生长、分化、凋亡,诱发恶性转化.迄今,国外对该病报道40余例,国内罕见~([1-2]).现报告我们发现的1例EMS,并进行文献复习.     

杀伤细胞免疫球蛋白样受体对造血干细胞移植的影响

自然杀伤(NK)细胞表面的杀伤细胞免疫球蛋白样受体(KIR),可通过与人类白细胞抗原(HLA)相互作用,影响NK细胞活性,进而影响异基因造血干细胞移植(allo-HSCT)的预后.在去T淋巴细胞allo-HSCT中,KIR/HLA错配的异源反应性NK细胞,在抗急性髓细胞白血病(AML)中发挥了重要作用;而未去T淋巴细胞allo-HSCT中,KIR/HLA错配对患者预后影响迥异.研究者提出,在自体造血干细胞移植(auto-HSCT)中也存在移植物抗白血病(GVL)效应,并且患者存在不同KIR对移植预后影响不同.笔者拟就KIR/HLA错配在造血干细胞移植中作用的研究进展进行综述.     

慢性髓系白血病异基因造血干细胞移植的研究进展

目前,异基因造血干细胞移植(allogeneic hematopoietic stem-cell transplantation,allo-HSCT)依旧是根治慢性髓系白血病(chronic myelogenous leukemia,CML)的唯一方法。随着HLA基因配型技术的改进、移植前后酪氨酸激酶抑制剂(tyrosine kinase inhibitor,TKI)的应用、术后免疫状态及融合基因监测的进步、术后并发症尤其是移植物抗宿主病(graft-versus-host disease,GVHD)的控制得到加强等,异基因造血干细胞移植在慢性粒细胞白血病治疗中正展现出更好的疗效,患者的生存质量也有了显著提高。本文将就近年来异基因造血干细胞移植及其相关支持技术在慢性髓系白血病治疗中最新研究进展作一综述。     

Acute myeloid leukemia with T lymphoblastic lymphoma: a case report and literature review

Acute myeloid leukemia (AML) with T lymphoblastic lymphoma (T-LBL) is a hematologic tumor of two origins, myeloid and lymphoblastic, and is relatively rare in the same patient. We report a rare case of AML with T-LBL. After the patient was diagnosed, he received standard chemotherapy, which decreased the primitive bone marrow cell percentage from 84% to 5%; however, the enlarged superficial lymph nodes showed no obvious change in size. Immunohistochemistry revealed the following: cluster of differentiation (CD)3 (+), CD5 (+), CD7 (+), transmission disequilibrium test (TDT) (+), myeloperoxidase (MPO) (−), and lysozyme (Lys) (−). The lymph node morphology and immunohistochemical results indicated T-LBL. Therefore, the final diagnosis was AML with T-LBL, with both diseases occurring independently and concurrently.     

混合表型急性白血病32例临床研究

目的 分析混合表型急性白血病(MPAL)的临床、生物学特征和预后.方法 总结2003年1月至2009年6月收治的32例MPAL患者资料,分析其骨髓细胞形态学、细胞免疫表型、细胞遗传学和分子生物学特点.32例患者采用了兼顾髓系、淋系的联合化疗方案治疗,2例在缓解期进行了异基因造血干细胞移植(allo-HSCT).对患者进行疗效和生存分析.结果 ①MPAL在急性白血病中的发生率为2.6%,32例MPAL患者中髓系和B系(M/B)表型16例(50.0%),髓系和T系(M/T)表型14例(43.8%),髓系、B、T三系(M/B/T)表型和B/T系表型各1例(3.1%).②CD34和HLA-DR阳性率分别为87.5%和62.5%.③异常核型发生率为70.0%(30例中21例),涉及Ph、11q23和复杂核型在内的结构重排和(或)数目异常.④32例MPAL患者总完全缓解(CR)率75.0%,2年总生存(OS)率和无病生存(DFS)率分别为14.8%和14.2%.M/B和M/T表型CR率分别为75.0%和71.4%.M/B和M/T表型患者的OS和DFS率比较差异无统计学意义.2例allo-HSCT患者均未复发,生存期延长.结论 MPAL是一类异质性的急性白血病亚型,其不良预后可能与染色体异常发生率高、CD34高表达和易于髓外浸润等因素有关.兼顾髓系、淋系的联合化疗和包括HSCT的强烈治疗可能有助于提高疗效.

Abstract：

Objective To investigate the clinical and biological characteristics and prognosis of mixed phenotype acute leukemia (MPAL). Methods Thirty two patients were diagnosed as MPAL by bone marrow examination, immunophenotyping, cytogenetic and molecular assay and were treated with combined chemotherapy regimens for both acute lymphoblastic and acute myeloid leukemia. Two cases were received allogeneic hematopoietic stem cell transplantation (allo-HSCT). Results ① The incidence of MPAL in acute leukemias was 2. 6%. There were 16 cases ( 50. 0% ) of mixed myeloid and B-lymphoid ( M/B), 14(43.8%) myeloid and T-lympboid ( M/T), one each (3.1%) of trilineage (M/B/T) and B- and T-lymphoid (B/T) phenotype. ② The positive rates of CD34 and HLA-DR were 87.5% and 62.5%, respectively.③Abnormal karyotypes were detected in 70.0% of 30 MPAL patients, which were structural and numerical abnormalities including t(9;22), 11q23 and complex karyotypes. ④ The total complete remission (CR) rate was 75.0% and the overall survival (OS) and disease-free survival (DES) at 2 years were 14. 8% and 14.2% respectively. The CR rates for M/B and M/T cases were 75.0% and 71.4% respectively. No statistical difference was observed in OS and DFS between M/B and M/T cases. Conclusions MPAL is a rare type of acute leukemia with a high heterogeneity. The unfavorable indicators of MPAL may be factors such as abnormal karyotypes, high expression of CD34 and extramedullary infiltration. Combined regimens and more intensive therapy including allo-HSCT might contribute to improving survival.     

18F-FDG PET/CT在成人Still病中的表现及其评估疾病活动度的作用

目的 :探讨18F-FDG PET/CT在成人Still病(Adult-onset still disease,AOSD)中的表现及其评估疾病活动度的作用。方法:选取确诊为AOSD并进行18F-FDG PET/CT检查的患者46例,收集其临床资料和PET/CT特点,分析AOSD患者的骨髓、脾脏、淋巴结标准化摄取值(Standardized uptake values,SUV)强度与实验室指标、系统评分之间的相关性。结果:AOSD的18F-FDG摄取主要发生于骨髓(100%,SUV强度:2.00±0.66)、脾(96%,SUV强度:1.70±0.54)及淋巴结(98%,SUV强度:2.67±1.38),此外18F-FDG摄取还可发生于心包、胸膜、咽部、颌下腺、腮腺、甲状腺、皮肤、关节、食管、贲门部位。骨髓SUV强度与疾病系统评分、血沉、C-反应蛋白、白细胞计数和中性粒百分数显著相关(r=0.445、0.376、0.323、0.439、0.524,P<0.05)。脾脏SUV强度与疾病系统评分、乳酸脱氢酶显著相关(r=0.424、0.347,P<0.05)。淋巴结SUV强度与疾病系统评分显著相关(r=0.356,P<0.05)。结论:AOSD在18F-FDG PET/CT上主要表现为骨髓、脾脏及淋巴结的FDG高摄取,18F-FDG PET/CT有助于监测疾病活动性。     

木村病临床超声影像及其他诊断学特征分析

目的探讨木村病的临床诊断学特征。 方法回顾性分析1例木村病患者的临床特征、实验室检验、基因检测、影像学特征、骨髓穿刺特征和病理学特征。 结果木村病患者白细胞计数11.65×10⁹/L,嗜酸性粒细胞绝对值3.12×10⁹/L,嗜酸性粒细胞占35.6%;红细胞沉降率32 mm/h;C反应蛋白20 mg/L;颈部淋巴结超声示左颈部可探及数个淋巴结,呈串珠样,皮质增厚,紧邻血管;胸腹部CT及纤维支气管镜检查未见明显异常。骨髓穿刺示骨髓增生活跃,以粒系和红系增生为主,巨核系无明显异常,嗜酸性细胞偏高。颈部淋巴结切除术中见颈前及外侧区多枚肿大淋巴结呈串珠样分布,部分融合成团,质韧,边界欠清,与周围组织粘连。组织病理学检查示淋巴组织大量增生,以淋巴滤泡增生为主,滤泡间小血管管壁增厚伴玻璃样变性,弥漫嗜酸性粒细胞浸润。组织免疫组化染色示：滤泡间区、滤泡散在的CD3和CD20(＋)、生发中心80%Ki-67(＋)、滤泡树突网CD21(＋)、生发中心Bcl-2(－)。 结论木村病是一种大量淋巴组织增生伴外周血嗜酸性粒细胞升高和血清IgE增高的慢性免疫性疾病。诊断需综合体格检查、实验室检验和影像学检查结果,明确诊断需组织病理学检查。     

急性微分化型髓细胞白血病转换为急性混合表型白血病一例诊断分析

目的 分析1例急性微分化型髓细胞白血病转换为不另作特定分类急性混合表型白血病的诊断过程,并探索其与其他急性微分化型髓细胞白血病和急性混合表型白血病病例的异同.方法 采用细胞涂片染色或化学染色方法对1例原发急性微分化型髓细胞白血病转换为不另作特定分类急性混合表型白血病病例进行细胞形态学分析;采用流式细胞术进行免疫表型分析;采用染色体G显带技术进行核型分析;应用RT-PCR技术进行融合基因的检测.并与2例急性微分化型髓细胞白血病和1例急性混合表型白血病进行实验室诊断结果比较,了解这一罕见的急性白血病发生转化的特征.结果 转换前急性微分化型髓细胞白血病在形态上表现为骨髓原始细胞占0.82,可见明显核仁,无Auer小体;免疫表型为造血相关抗原CD38和HLA-DR阳性,部分髓系抗原(CD13、CD56和CD11b)阳性,淋系抗原CD7阳性;其他髓系抗原(MPO、CD33和CD15)阴性,B系抗原(CD79a、CD19和CD22)阴性,T系抗原(胞内CD3、CD4和CD8)阴性.而转换后不另作特定分类急性混合表型白血病在形态上表现为骨髓原始细胞极度增生,占0.42,嗜酸粒细胞增多,嗜碱粒细胞可见;免疫表型为造血相关抗原CD38和HLA-DR阳性,髓系抗原(MPO和CD13)阳性,B系抗原(CD19和CD79a)阳性,T系抗原(胞内CD3)阳性,淋系抗原CD7阳性.对照组白血病具有典型的形态学和免疫表型特点,均未见异常染色体核型和融合基因.结论 该病例诊断复杂,临床少见,综合分析急性微分化型髓细胞白血病和不另作特定分类急性混合表型白血病的实验室特征对确诊十分重要,而免疫表型的变化是关键.

Abstract：

Objective To analyze the diagnostic process of a rare case of acute myeloid leukemia with minimal differentiation undergoing a lineage switch to mixed phenotype acute leukemia, NOS-rare types,and to investigate its difference from other acute myeloid leukemia and mixed phenotype acute leukemia. Methods Following tests were performed on the patient with switched mixed phenotype acute leukemia and three control leukemia patients ( including two acute myeloid leukemia with minimal differentiation and one mixed phenotype acute leukemia ). Cell morphology was analyzed by bone marrow smear and related cell chemical staining. Immunophenotyping of bone marrow was performed by flow cytometry ( FCM ). G-banding technique was used for karyotype analysis and RT-PCR was used for fusion gene detection. All the laboratory data of the switched patient were compared to that of three control patients in order to reveal the characteristics of such a rare phenotype switch in acute leukemia. Results Before switching, the morphology of acute myeloid leukemia with minimal differentiation demonstrated 0.82 blasts occurring in bone marrow, distinct nucleoli and absence of Auer rods. Blast cells expressed hematopoieticassociated antigens ( CD38, HLA-DR ), myeloid antigens ( CD13, CD56, CD11b ) and CD7. And these blasts were negative for MPO, CD33, CD15, CD79, CD19, CD22, cytoplasmic CD3, CD4 and CD8. After switching, 0. 42 blasts were found in bone marrow, showed eosinophilia and presence of basophile. Blast cells expressed hematopoietic-associated antigens ( CD38, HLA-DR ), myeloid antigens ( MPO, CD13 ),lymphoid antigens ( CD19, CD79a ,cytoplasmic CD3, and CD7 ). The control group showed typical morphology and immunophenotyping. No abnormal karyotype and fusion gene were detected. Conclusions It is a rare and complicated case that acute myeloid leukemia with minimal differentiation switched to mixed phenotype acute leukemia, NOS-rare types. The laboratory features, especially the change of immunophenotyping play an important role in the diagnosis.     

流式细胞术检测异基因造血干细胞移植后多形性淋巴细胞增殖性疾病——附二例报告

目的 研究流式细胞术检测在异基因造血干细胞移植(allo-HSCT)后多形性淋巴细胞增殖性疾病诊断中的作用.方法 采用多色流式细胞术诊断allo-HSCT后多形性淋巴细胞增殖性疾病.结果 2例ailo-HSCT患者分别于移植后46 d(+46 d)和+50 d出现高热,多处淋巴结肿大,抗炎治疗无效,骨髓EB病毒DNA水平升高,经流式细胞术检测发现外周血多群轻链限制性单克隆B细胞和(或)浆细胞.诊断为移植后多形性淋巴细胞增殖性疾病.经免疫抑制减量、抗病毒、使用利妥昔单抗、输细胞毒性T淋巴细胞治疗后,经流式细胞术随访监测外周血和(或)骨髓标本.2例患者的B细胞克隆均消失,但是单克隆浆细胞持续存在或者新出现的克隆.1例患者2周后死亡;另1例患者仍在治疗中,外周血未见B细胞和浆细胞,骨髓未见B细胞,可见单克隆浆细胞.结论 使用流式细胞术可以有效诊断移植后多形性淋巴细胞增殖性疾病,并进行病情监测.随访过程中,骨髓标本可能比外周血标本敏感.allo-HSCT患者如果没有淋巴结活检,通过检测外周血也可以发现B细胞异常.     

Hemophagocytic lymphohistiocytosis secondary to composite lymphoma: Two case reports

BACKGROUNDHemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disease caused by inherited pathogenic mutations and acquired dysregulations of the immune system. Composite lymphoma is defined as two or more morphologically and immunophenotypically distinct lymphomas that occur in a single patient. Here, we report two cases of HLH secondary to composite lymphoma with mixed lineage features of T- and B-cell marker expression both in the bone marrow and lymph nodes in adult patients.CASE SUMMARYTwo patients were diagnosed with HLH based on the occurrence of fever, pancytopenia, lymphadenopathy, splenomegaly, hemophagocytosis and hyperferritinemia. Immunohistochemical staining of the axillary lymph node and bone marrow in case 1 showed typical features of combined B-cell and T-cell lymphoma. In addition, a lymph node gene study revealed rearrangement of the T-cell receptor chain and the immunoglobulin gene. Morphology and immunohistochemistry studies of a lymph node biopsy in case 2 showed typical features of T cell lymphoma, but immunophenotyping by flow cytometry analysis of bone marrow aspirate showed B cell lymphoma involvement. The patients were treated with high-dose methylprednisolone combined with etoposide to control aggressive HLH progression. The patients also received immunochemotherapy with the R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) regimen immediately after diagnosis. Both patients presented with highly aggressive lymphoma, and died of severe infection or uncontrolled HLH.CONCLUSIONWe present two rare cases with overwhelming hemophagocytosis along with composite T- and B-cell lymphoma, which posed a diagnostic dilemma. HLH caused by composite lymphoma was characterized by poor clinical outcomes.     

用流式细胞术从B淋巴增殖性疾病中鉴别脾边缘带淋巴瘤

脾边缘带淋巴瘤（Splenicmarginalzonelymphoma,SMZL）是一种相对少见的原发于脾脏的惰性B淋巴增殖性疾病,表现为外周血淋巴细胞计数和／或比例增高、脾大,而外周浅表淋巴结往往不大,需要应用诊断性脾切除病理检查得以确诊,但患者多数不能接受,早期诊断困难。本研究探索以流式细胞术（flowcytometry,FCM）为主的诊断途径的可行性。选取6例疑诊SMZL患者为研究对象,同时以10名骨髓健康供者及确诊的10例慢性淋巴细胞白血病（CLL）、3例毛细胞白血病（HCL）、3例淋巴浆细胞淋巴瘤／华氏巨球蛋白血症（LPL／wM）初诊患者为对照。对所有患者及对照组进行骨髓FCM免疫分型,所选抗体组合包括CD45、CD5、CD10、CD19、CD20、CD22、CD23、CD25、CD103、CD11c、CD123、κ、λ、CyclinD1等,同时结合骨髓细胞形态学检查。结果表明：6例疑诊sMzL患者表现为淋巴细胞增高和脾大。因外周淋巴结无肿大6例患者均未行淋巴结活检,仅1例患者行病理诊断性脾切除。经骨髓FCM免疫表型分析,除健康供者外均可发现骨髓中存在不同程度的异常成熟B细胞克隆,并通过CD5、CD10表达与否结合其他表型进一步与其他B细胞肿瘤鉴别。结果6例SMZL患者均CDl9＋、CD20＋,而CD10-,4例患者CD5-,2例CD5＋,而CD23、CD38、ZAP-70、CD11c、CD103、CD123、CyclinD1均不表达。骨髓细胞形态学检查可见有短绒毛的异常淋巴细胞,结合临床特征将6例患者诊断为SMZL。1例患者因合并脾功能亢进需要行脾切除,术后病理证实该病。10例CLL主要表达CD5、CD23之外;在3例HCL除表达CD11c、CD103、CD123,形态学上更有典型的”毛”;3例LPL／WM具有轻链限制性表达、IgM显著升高、浆样淋巴细胞增多的特点。结论：FCM免疫表型分析结合骨髓形态学可作为临床诊断SMZL有力的工具。