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.

Bendamustine and rituximab as frontline therapy in extranodal marginal zone lymphoma:a single-institution experience

Extranodal marginal zone lymphoma(EMZL)encompasses 70%of cases of marginal zone lymphoma.Frontline bendamustine and rituximab(BR)were derived from trials involving other indolent non-Hodgkin’s lymphomas.Only one trial has evaluated frontline BR prospectively in EMZL.This retrospective study reports outcomes among EMZL patients receiving frontline BR.Twenty-five patients were included with a median age of 69 years(40–81).Five(20.0%)patients had stage Ⅰ/Ⅱ disease,and 20(80.0%)had stage Ⅲ/Ⅳ disease.The median number of cycles was 6.0(3.0–6.0).Maintenance rituximab was administered to 10(41.7%)individuals.Overall response rate(ORR)was 100.0%(60.0%complete response,40.0%partial response).Medians of overall survival and progression-free survival were not reached.The estimated 2-year progression-free survival was 85.2%and overall survival was 100.0%.Four(16.6%)patients had infections related to treatment;3(12.0%)transformed to diffuse large B-cell lymphoma;5(20.8%)had a relapse or progression of EMZL;and 3(12.0%)died unrelated to BR.BR is an efficacious and well-tolerated front-line regimen for EMZL with response data consistent with existing literature.

Transformation of marginal zone lymphoma into high-grade B-cell lymphoma expressing terminal deoxynucleotidyl transferase:A case report

BACKGROUND High-grade B-cell lymphoma(HGBL)is an unusual malignancy that includes myelocytomatosis viral oncogene(MYC),B-cell lymphoma-2(BCL-2),and/or BCL-6 rearrangements,termed double-hit or triple-hit lymphomas,and HGBL-not otherwise specific(HGBL-NOS),which are morphologically characteristic of HGBL but lack MYC,BCL-2,or BCL-6 rearrangements.HGBL is partially transformed by follicular lymphoma and other indolent lymphoma,with few cases of marginal zone lymphoma(MZL)transformation.HGBL often has a poor prognosis and intensive therapy is currently mainly advocated,but there is no good treatment for these patients who cannot tolerate chemotherapy.CASE SUMMARY We reported a case of MZL transformed into HGBL-NOS with TP53 mutation and terminal deoxynucleotidyl transferase expression.Gene analysis revealed the gene expression profile was identical in the pre-and post-transformed tissues,suggesting that the two diseases are homologous,not secondary tumors.The chemotherapy was ineffective and the side effect was severe,so we tried combination therapy including venetoclax and obinutuzumab.The patient tolerated treatment well,and reached partial response.The patient had recurrence of hepatocellular carcinoma and died of multifunctional organ failure.He survived for 12 months after diagnosis.CONCLUSION Venetoclax combined with obinutuzumab might improve the survival in some HGBL patients,who are unsuitable for chemotherapy.

Laparoscopic splenectomy for treatment of splenic marginal zone lymphoma

AIM: To investigate the short-term and long-term ef-ficacy and safety of laparoscopic splenectomy (LS) for treatment of splenic marginal zone lymphoma (SMZL). METHODS: A total of 18 continuous patients who were diagnosed with SMZL and underwent LS in our department from 2008 to 2012 were reviewed. The perioperative variables and long-term follow-up were evaluated. To evaluate the efficacy and safety of this procedure better, we also included 34 patients with liver cirrhosis who underwent LS, 49 patients with immune thrombocytopenia (ITP) who underwent LS, and 20 patients with SMZL who underwent open splenectomy (OS). The results observed in the different groups were compared.RESULTS: No differences were found in the sex and Child-Pugh class of the patients in SMZL-LS, SMZL-OS, ITP, and liver cirrhosis groups. The splenic length of the patients in the SMZL-LS group was similar to that in the SMZL-OS and liver cirrhosis groups but significantly longer than in the ITP group. The SMZL-LS group had a significantly longer operating time compared with the SMZL-OS, ITP, and liver cirrhosis groups, and the SMZL-LS group exhibited significantly less blood loss compared with the SMZL-OS group. No difference was found in the length of the postoperative hospital stay between the SMZL-LS, SMZL-OS, ITP, and liver cirrhosis-LS groups. After surgery, 6 (33.3%) SMZL-LS patients suffered slight complications. During mean fol-low-up periods of 13.6 and 12.8 mo, one patient from the SMZL-LS group and two from the SMZL-OS group died as a result of metastasis after surgery. None of the ITP and liver cirrhosis patients died. CONCLUSION: LS should be considered a feasible and safe procedure for treatment of SMZL in an effort to improve the treatment options and survival of patients.

Splenectomy with chemotherapy vs surgery alone as initial treatment for splenic marginal zone lymphoma

AIM： To evaluate the clinical characteristics of splenic marginal-zone lymphoma （SMZL） following antigen expression and the influence of therapeutic approaches on clinical outcome and overall survival （OS）.METHODS： A total of 30 patients with typical histological and immunohistochemical SMZL patterns were examined. Splenectomy plus chemotherapy was applied in 20 patients, while splenectomy as a single treatment-option was performed in 10 patients. Prognostic factor and overall survival rate were analyzed.RESULTS： Complete remission （CR） was achieved in 20 （66.7%）, partial remission （PR） in seven （23.3%）, and lethal outcome due to disease progression occurred in three （10.0%） patients. Median survival of patients with a splenectomy was 93.0 mo and for patients with splenectomy plus chemotherapy it was 207.5 mo （Log rank = 0.056, P 〉 0.05）. Time from onset of first symptoms to the beginning of the treatment （mean 9.4 too） was influenced by spleen dimensions, as measured by computerized tomography and ultra-sound （t = 2.558, P = 0.018）. Strong positivity （＋＋＋） of CD20 antigen expression in splenic tissue had a positive influence on OS （Log rank = 5.244, P 〈 0.05）. The analysis of factors interfering with survival （by the Kaplan-Meier method） revealed that gender, general symptoms, clinical stage, and spleen infiltration type （nodular vs diffuse） had no significant （P 〉 0.05） effects on the OS. The expression of other antigens （immunohistochemistry） also had no effect on survival-rate, as measured by a χ^2 test （P 〉 0.05）.CONCLUSION： Initial splenectomy combined with chemotherapy has been shown to be beneficial due to its advanced remission rate/duration; however, a larger controlled clinical study is required to confirm our findings.

Primary mucosal-associated lymphoid tissue extranodal marginal zone lymphoma of the bladder from an imaging perspective: A case report

BACKGROUND Mucosal-associated lymphoid tissue extranodal marginal zone(MALT)lymphoma is a low-grade tumor that rarely occurs in the urinary bladder.There is currently no consensus on the common imaging findings or most appropriate treatment in MALT lymphoma in the urinary bladder due to the limited number of reports.CASE SUMMARY A 48-year-old woman was admitted to the hospital with a 1-year history of macroscopic hematuria.Imaging showed a large homogeneous mass with an unclear boundary and an irregular morphology in the bladder.The mass had an abundant blood supply.For further diagnosis,transurethral cystoscopic biopsy and bone marrow biopsy was performed,and the patient was finally diagnosed with primary MALT lymphoma of the bladder.R-CHOP chemotherapy was carried out.After three cycles of chemotherapy,the mass disappeared and the bladder wall thickness was only 4 mm,which indicated excellent therapeutic response to the chemotherapy.To date,the patient remains asymptomatic and she visits our hospital regularly for the completion of the remaining chemotherapy cycles.CONCLUSION Primary MALT lymphoma of the bladder is rare,and there are certain characteristics in the ultrasonographic findings.Imaging findings play an important role in evaluating the therapeutic efficacy and are critical during long-term follow-up after therapy.

Secondary light chain amyloidosis with Waldenstr?m’s macroglobulinemia and intermodal marginal zone lymphoma:A case report

BACKGROUND The co-existence of Waldenstr?m’s macroglobulinemia(WM) with internodal marginal zone lymphoma(INMZL) is rare and often associated with poor prognosis.CASE SUMMARY We present a Chinese female patient who developed secondary light chain amyloidosis due to WM and INMZL and provides opinions on its systemic treatment.A 65-year-old woman was diagnosed with WM 6 years ago and received Bruton tyrosine kinase inhibitor monotherapy for two years.Her INMZL was confirmed due to left cervical lymphadenopathy.The patient presented with oedema in both lower limbs one year ago,and was diagnosed with secondary light chain amyloidosis.Treatment with the BC regimen(rituximab 375 mg/m~2 monthly for 6-8 courses,and bendamustine 90 mg/m~2 per day × 2,monthly for six courses) was initiated,but not tolerated due to toxic side effects.Bortezomibbased therapy was given for two months,including bortezomib,dexamethasone,and zanubrutinb.Oedema in both lower limbs was relieved and treatment efficacy was evaluated as partial remission.CONCLUSION A detailed clinical evaluation and active identification of the aetiology are recommended to avoid missed diagnosis and misdiagnosis.

Sclerotic marginal zone lymphoma:A case report

BACKGROUND Marginal zone lymphoma(MZL)is an indolent non-Hodgkin B cell lymphoma with various architectural pattern including perifollicular,follicular colonization,nodular,micronodular,and diffuse patterns.A sclerotic variant has not been previously reported and represents a diagnostic pitfall.CASE SUMMARY A 66-year-old male developed left upper extremity swelling.Chest computed tomography(CT)in September 2020 showed 14 cm mass in left axilla.Needle core biopsy of axillary lymph node showed sclerotic tissue with atypical B lymphoid infiltrate but was non-diagnostic.Excisional biopsy was performed for diagnosis and showed extensive fibrosis and minor component of infiltrating B cells.Flow cytometry showed a small population of CD5-,CD10-,kappa restricted B cells.Monoclonal immunoglobulin heavy chain and light chain gene rearrangement were identified.Upon being diagnosed with MZL,patient was treated with rituximab,cyclophosphamide,doxorubicin,vincristine,and prednisone and achieved complete remission by positron emission tomography/CT.CONCLUSION This is an important case report because by morphology this case could have easily been overlooked as non-specific fibrosis with chronic inflammation representing a significant diagnostic pitfall.Moreover,this constitutes a new architectural pattern.While sclerotic lymphomas have rarely been described(often misdiagnosed as retroperitoneal fibrosis),we do not know of any cases describing this architectural presentation of MZL.

Splenic Marginal Zone Lymphoma in a Patient with Positive Hepatitis B Virus Serology

We present a case in which an elderly woman diagnosed with a splenic marginal zone lymphoma (MZL) was found to have positive Hepatitis B serology. Link with Hepatitis C virus is well documented but reports of association of Hepatitis B virus (HBV) with splenic marginal zone lymphoma are still emerging. A 69-year-old lady presented with weight loss, pancytopenia and marked splenomegaly. Prior to commencing treatment, Hepatitis B serology confirmed Hepatitis B infection. She was treated with Chlorambucil along with anti-hepatitis B prophylaxis and HBV PCR monitoring. She had an excellent response to treatment with resolution of symptoms and splenomegaly. This case highlights the importance of testing for hepatitis B serology in patients diagnosed with splenic MZLs as causative agent. Although the association between HCV is well documented in the literature, a relationship between HBV may also be important. Also, chemotherapy +/- Rituximab for splenic MZL is associated with the reactivation of latent infections;hence providing prophylactic cover for pre-existing latent HBV infection may be required to prevent reactivation as in this case.

Role of Helicobacter pylori in gastric mucosa-associated lymphoid tissue lymphomas

Mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent extranodal marginal zone B-cell lymphoma, originating in acquired MALT that is induced in mucosal barriers as part of a normal adaptive immune response to a chronic immunoinflammatory stimulus, most notably chronic infection by Helicobacter pylori (H. pylori). This antigenic stimulation initially leads to lymphoid hyperplasia; the acquisition of additional genetic aberrations culminates in the activation of intracellular survival pathways, with disease progression due to proliferation and resistance to apoptosis, and the emergence of a malignant clone. There are descriptions of MALT lymphomas affecting practically every organ and system, with a marked geographic variability partially attributable to the epidemiology of the underlying risk factors; nevertheless, the digestive system (and predominantly the stomach) is the most frequently involved location, reflecting the gastrointestinal tract’s unique characteristics of contact with foreign antigens, high mucosal permeability, large extension and intrinsic lymphoid system. While early-stage gastric MALT lymphoma can frequently regress after the therapeutic reversal of the chronic immune stimulus through antibiotic eradication of H. pylori infection, the presence of immortalizing genetic abnormalities, of advanced disease or of eradication-refractoriness requires a more aggressive approach which is, presently, not consensual. The fact that MALT lymphomas are rare neoplasms, with a worldwide incidence of 1-1.5 cases per 105 population, per year, limits the ease of accrual of representative series of patients for robust clinical trials that could sustain informed evidence-based therapeutic decisions to optimize the quality of patient care.

Management of gastric mucosa-associated lymphoid tissue lymphoma in patients with extra copies of the MALT1 gene

AIM To identify the clinical features of gastric mucosaassociated lymphoid tissue(MALT) lymphoma with extra copies of MALT1.METHODS This is a multi-centered,retrospective study. We reviewed 146 patients with MALT lymphoma in the stomach who underwent fluorescence in situ hybridization analysis for t(11;18) translocation. Patients were subdivided into patients without t(11;18) translocation or extra copies of MALT1(Group A,n = 88),patients with t(11;18) translocation(Group B,n = 27),and patients with extra copies of MALT1(Group C,n = 31). The clinical background,treatment,and outcomes of each group were investigated.RESULTS Groups A and C showed slight female predominance,whereas Group B showed slight male predominance. Mean ages and clinical stages at lymphoma diagnosis were not different between groups. Complete response was obtained in 61 patients in Group A(69.3%),22 in Group B(81.5%),and 21 in Group C(67.7%). Helicobacter pylori(H. pylori) eradication alone resulted in complete remission in 44 patients in Group A and 13 in Group C. In Group B,14 patients underwent radiotherapy alone,which resulted in lymphoma disappearance. Although the difference was not statistically significant,event-free survival in Group C tended to be inferior to that in Group A(P = 0.10).CONCLUSION Patients with t(11;18) translocation should be treated differently from others. Patients with extra copies of MALT1 could be initially treated with H. pylori eradication,similar to patients without t(11;18) translocation or extra copies of MALT1.

Relationships between lymphomas linked to hepatitis C virus infection and their microenvironment

The relationships between lymphomas and their microenvironment appear to follow 3 major patterns:(1)an independent pattern;(2)a dependent pattern on deregulated interactions;and(3)a dependent pattern on regulated coexistence.Typical examples of the third pattern are hepatitis C virus(HCV)-associated marginal zone lymphomas(MZLs)and mucosa-associated lymphoid tissue lymphomas.In these lymphomas,a regulated coexistence of the malignant cells and the microenvironmental factors usually occurs.At least initially,however,tumor development and cell growth largely depend on external signals from the microenvironment,such as viral antigens,cytokines,and cell-cell interactions.The association between HCV infection and B-cell lymphomas is not completely defined,although this association has been demonstrated by epidemiological studies.MZL and diffuse large B-cell lymphoma are the histotypes most frequently associated with HCV infection.Many mechanisms have been proposed for explaining HCV-induced lymphomagenesis;antigenic stimulation by HCV seems to be fundamental in establishing B-cell expansion as observed in mixed cryoglobulinemia and in B-cell lymphomas.Recently,antiviral treatment has been proved to be effective in the treatment of HCV-associated indolent lymphomas.Importantly,clinically responses were linked to the eradication of the HCV-RNA,providing a strong argument in favor of a causative link between HCV and lymphoproliferation.

HCV infection, B-cell non-Hodgkin's lymphoma and immunochemotherapy: Evidence and open questions

There is plenty of data confirming that hepatitis C virus (HCV) infection is a predisposing factor for a B-cell non-Hodgkin's lymphoma (B-NHL) outbreak, while relatively few reports have addressed the role of HCV in affecting B-NHL patients' outcome. HCV infection may influence the short-term outcome of B-NHL because of the emergence of severe hepatic toxicity (HT) during immunochemotherapy. Furthermore, the long term outcome of HCV-related liver disease and patients' quality of life will possibly be affected by Rituximab maintenance, multiple-lines of toxicity during chemotherapy and hematopoietic stem cell transplantation. In this review, data dealing with aggressive and low-grade B-NHL were separately analyzed. The few retrospective papers reporting on aggressive B-NHL patients showed that HCV infection is a risk factor for the outbreak of severe HT during treatment. This adverse event not infrequently leads to the reduction of treatment density and intensity. Existing papers report that low-grade B-NHL patients with HCV infection may have a more widespread disease, more frequent relapses or a lower ORR compared to HCV-negative patients. Notwithstanding, there is no statistical evidence that the prognosis of HCV-positive patients is inferior to that of HCV-negative subjects. HCV-positive prospective studies and longer follow-up are necessary to ascertain if HCV-positive B-NHL patients have inferior outcomes and if there are long term sequels of immunochemotherapies on the progression of liver disease.

Antiviral therapy of hepatitis C as curative treatment of indolent B-cell lymphoma

The association of hepatitis C virus(HCV) and B-cell non-Hodgkin lymphomas(NHL) has been highlighted by several epidemiological and biological insights; however the most convincing evidence is represented by interventional studies demonstrating the capability of antiviral treatment(AT) with interferon(IFN) with or without ribavirin to induce the regression of indolent lymphomas, especially of marginal-zone origin. In the largest published retrospective study(100 patients) the overall response rate(ORR) after first-line IFN-based AT was 77%(44% complete responses) and responses were sustainable(median duration of response 33 mo). These results were confirmed by a recent metaanalysis on 254 patients, demonstrating an ORR of 73%. Moreover this analysis confirmed the highly significant correlation between the achievement of viral eradication sustained virological response(SVR) and hematological responses. Two large prospective studies demonstrated that AT is associated with improved survival and argue in favor of current guidelines' recommendation of AT as preferential first-line option in asymptomatic patients with HCV-associated indolent NHL. The recently approved direct-acting antiviral agents(DAAs) revolutionized the treatment of HCV infection, leading to SVR approaching 100% in all genotypes. Very preliminary data of IFN-free DAAs therapy in indolent HCV-positive NHL seem to confirm their activity in inducing lymphoma regression.

T(11;18)(q21;q21)-positive gastrointestinal MALT lymphomas are heterogeneous with respect to the V_H gene mutation status

AIM: To investigate how t(11;18)(q21;q21)-positive gastrointestinal MALT lymphomas relate to other marginal zone lymphomas with respect to the somatic mutation pattern of the VH genes and the expression of the marker CD27. METHODS: The VH gene of 7 t(11;18)(q21;q21)-positive gastrointestinal MALT lymphomas was amplif iedby PCR using family specif ic VH primers and a consensus JH primer. PCR products were sequenced and mutation analysis of the CDR and the FR regions was performed. All cases were immunostained for CD27. RESULTS: One case showed unmutated VH genes while the others showed mutated VH genes with mutation frequencies ranging from 1.3 to 14.7% and with evidence of antigen selection in 2 cases. These data suggest that the translocation t(11;18)(q21;q21) can target either B-cells at different stages of differentiation or naive B-cells that retain the capacity to differentiate upon antigen stimulation. All cases but one displayed weak to strong CD27 expression which did not correlate with the VH gene mutation status. CONCLUSION: t(11;18)(q21;q21)-positive gastro- intestinal MALT lymphomas are heterogeneous with respect to the VH mutation status and CD27 is not a marker of somatically mutated B-cells.

脑膜结外边缘区淋巴瘤误诊为亚急性期硬膜下血肿1例报告

1病例女,72岁,反复头晕数月,3天前不慎摔倒来诊。查体和实验室检查均未发现明显异常。2021年3月19日门诊CT平扫(图1)发现右额顶部颅骨内板下见一新月形稍高密度影,邻近脑实质受压移位。次日头颅MR平扫(图2)示右额顶部颅骨内板下见一弧形T_(1)WI稍低信号、T_(2)WI等/稍低信号影,DWI呈稍高信号,ADC值减低;右额顶叶部分脑沟内见少许线性T_(2)WI/flair稍高信号影。影像拟诊:(1)亚急性期硬膜下血肿;(2)脑膜炎可能。2021年4月3日CT复查(图3)提示右额顶部颅骨内板下新月形稍高密度影同前。患者头晕、肢体乏力症状加重,并出现头痛。2021年12月28日CT(图4)发现右额顶部颅骨内板下新月形稍高密度影范围较前扩大。2021年12月31日MR平扫及增强(图5)提示右额顶部颅骨内板下新月形异常信号影较前扩大,增强后均匀中-明显强化,考虑恶性肿瘤;右额顶叶部分脑沟内线性T_(2)WI/flair稍高信号影同前;右额顶叶新发斑片状T_(1)WI稍低信号、T_(2)WI稍高信号影,DWI呈等信号,增强后无强化,考虑肿瘤浸润可能。患者临床症状逐渐加重故行脑病损切除术。

原发性皮肤边缘区B细胞淋巴瘤1例

报告原发性皮肤边缘区B细胞淋巴瘤1例。患者男,23岁,因背部红色结节5年就诊。皮肤科检查:背部多发大小不一红色结节,约5 mm×5 mm~10 mm×15 mm,表面光滑,边界清,未见明显鳞屑,质地较软,无压痛。皮损组织病理示:真皮层及皮下组织淋巴组织增生呈结节状分布,部分滤泡萎缩,其周围及小汗腺周围可见小-中等的中心细胞样及单核细胞样细胞围绕,局部侵及皮下脂肪组织。免疫组化示:CD20(+)、CD21(FDC网+)、CD23(FDC网+)、kappa(+)、Bcl-2(+)、CD43(部分+)、Ki-67(10%+),CD3、CD4、CD5、CD8、Lambda、Bcl-6、Cyclin-D1均阴性。诊断:原发性皮肤边缘区B细胞淋巴瘤。患者放弃治疗,1年后失访。

伴血象异常的脾边缘区淋巴瘤的临床特点

本文探讨伴有血细胞计数异常的脾边缘区淋巴瘤(SMZL)的临床特点。回顾性地分析了19例因血细胞计数异常就诊并被确诊为SMZL患者的资料,其中7例根据脾脏病理确诊,12例不能接受脾切除术者应用脾B细胞淋巴瘤研究组的最低诊断标准进行诊断。结果表明,5例(26.3%)患者白细胞≥10.0×109/L、6例(31.6%)<4.0×109/L;14例(73.7%)血红蛋白<120 g/L;11例(57.9%)血小板<100.0×109/L;14例(73.8%)患者具有血细胞减少,其中一系血细胞减少6例(31.6%),两系血细胞减少4例(21.1%),三系血细胞减少4例(21.1%);19例均有骨髓累及;10例(52.6%)检出带绒毛的淋巴细胞;淋巴瘤细胞在骨髓内呈现包括窦内浸润的多种分布特点;单克隆免疫球蛋白升高者占56.3%(9/16);存在自身免疫现象者占80%(12/15);4例进行单纯脾切除术,血象均无改善;1例接受脾切除术联合化疗获得部分缓解;11例接受含有利妥昔单抗的治疗,总体反应率100.0%,其中9例(81.8%)获得完全缓解。结论:伴有血细胞计数异常的SMZL以血细胞减少、骨髓高累及率和自身免疫现象为多见,含利妥昔单抗的治疗具有较好的疗效。

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(Splenic marginal zone lymphoma,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(flow cytometry,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、Cyclin D1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CD19+、CD20+,而CD10-,4例患者CD5-,2例CD5+,而CD23、CD38、ZAP-70、CD11c、CD103、CD123、Cyclin D1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。

脾边缘带淋巴瘤3例报告及文献复习

目的总结脾边缘带淋巴瘤的临床特点、组织病理学特征、诊断、治疗方法及预后。方法回顾分析我院3例脾边缘带淋巴瘤患者的临床和随诊资料,并对相关文献进行复习。结果3例患者发病年龄49～71岁,从出现症状到明确诊断时间为3～11个月。3例发病时均有脾大,1例出现脾功能亢进造成的全血细胞减少,2例出现发热等B组症状,2例有结外受累。3例均为脾切除后经病理明确诊断,2例化疗后随诊仍为完全缓解,1例化疗结束后10个月复发。结论脾边缘带淋巴瘤是一种惰性的淋巴增殖性疾病,中位生存期长,65%～78%患者的诊断后生存期可超过5年,故明确诊断后可采用相对保守的治疗措施。