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1.
Primary splenic involvement in lymphoid neoplasms is rare and the clinicopathologic features of splenic lymphoma are not well described compared to nodal non-Hodgkin's lymphoma (NHL). Here we characterized splenic lymphomas in an human T lymphotropic virus type-I (HTLV-I) endemic area of Japan. To assess the pattern of splenic involvement and evaluate prognosis, we reviewed 27 specimens consisting of 26 splenectomies and one necropsy, which were classified using REAL classification. Cases were divided into primary splenic lymphoma in 11 patients and secondary in 16 patients. The incidence of primary splenic lymphoma was 0.3% (11 of approximately 4,000 malignant lymphomas). Primary splenic lymphomas included 7 diffuse large B cell lymphoma (DLBL), 2 follicular lymphomas (FL), and 1 each of splenic marginal zone lymphoma (SMZL) and anaplastic large cell lymphoma (ALCL). Secondary splenic lymphomas included 6 DLBL, 4 mantle cell lymphoma (MCL), 2 FL, 2 Hodgkin's disease (HD), 1 each of hairy cell leukemia and ALCL. Gross examination showed two patterns of splenic involvement; solid type (formation of large nodular mass, n=16) and disseminated type (multiple nodules with diffuse infiltration but no large nodular formation, n=10). The type could not be determined in one case. Most solid types were DLBL or FL, while MCL was of the disseminated type. Immunohistochemistry showed all but each 2 cases of ALCL and HD were of B lineage. Follow-up of 26 patients indicated that all but one patient with primary lymphoma were still alive (range, 1-89 months) and 8 of 15 patients with secondary lymphomas died due to the progression of malignant lymphoma; the survival rate at 2 years was 50% in these patients. No elevation of anti-HTLV-I antibody was found. In situ hybridization for Epstein-Barr virus (EBV) showed no reactivity of lymphoma cells, although a few small lymphocytes were positive for EBV. Hepatitis C virus was observed in 6 of 20 (30%) patients examined and 4 of 11 (36%) cases of primary splenic lymphoma. Our findings indicate that patients with primary splenic lymphoma have a favorable prognosis after splenectomy.  相似文献   

2.
Clonally related composite lymphomas of Hodgkin's lymphoma (HL) and Non-Hodgkin's lymphoma (NHL) represent models to study the multistep transformation process in tumorigenesis and the development of two distinct tumors from a shared precursor. We analyzed six such lymphomas for transforming events. The HLs were combined in two cases with follicular lymphoma (FL), and in one case each with B-cell chronic lymphocytic leukemia, splenic marginal zone lymphoma, mantle cell lymphoma (MCL) and diffuse large B-cell lymphoma (DLBCL). In the HL/FL and HL/MCL combinations, BCL2/IGH and CCND1/IGH translocations, respectively, were detected in both the HL and NHL. No mutations were found in the tumor suppressor genes FAS, NFKBIA and ATM. The HL/DLBCL case harbored clonal replacement mutations of the TP53 gene on both alleles exclusively in the DLBCL. In conclusion, we present the first examples of molecularly verified IgH-associated translocations in HL, which also show that BCL2/IGH or CCND1/IGH translocations can represent early steps in the pathogenesis of composite HL/FL or HL/MCL. The restriction of the TP53 mutations to the DLBCL in the HL/DLBCL case exemplifies a late transforming event that presumably happened in the germinal center and affected the fate of a common lymphoma precursor cell towards development of a DLBCL.  相似文献   

3.

Background

Most malignant lymphomas in HIV-patients are caused by reactivation of EBV-infection. Some lymphomas have a very rapid fulminant course. HHV-8 has also been reported to be a cause of lymphoma. The role of CMV in the development of lymphoma is not clear, though both CMV and HHV-8 have been reported in tissues adjacent to the tumour in Burkitt lymphoma patients. Here we present a patient with asymptomatic HIV infection, that contracted a primary cytomegalovirus (CMV) infection and human herpes virus 8 (HHV-8) infection. Three weeks before onset of symptoms the patient had unprotected sex which could be possible source of his CMV and also HHV-8 infection He deteriorated rapidly and died with a generalized anaplastic large cell lymphoma (ALCL).

Methods

A Caucasian homosexual male with asymptomatic human immunodeficiency virus (HIV) infection contracted a primary cytomegalovirus (CMV) infection and human herpes virus 8 (HHV-8) infection. He deteriorated rapidly and died with a generalized anaplastic large cell lymphoma (ALCL). Clinical and laboratory records were compiled. Immunohistochemistry was performed on lymphoid tissues, a liver biopsy, a bone marrow aspirate and the spleen during the illness and at autopsy. Serology and PCR for HIV, CMV, EBV, HHV-1–3 and 6–8 was performed on blood drawn during the course of disease.

Results

The patient presented with an acute primary CMV infection. Biopsies taken 2 weeks before death showed a small focus of ALCL in one lymph node of the neck. Autopsy demonstrated a massive infiltration of ALCL in lymph nodes, liver, spleen and bone marrow. Blood samples confirmed primary CMV- infection, a HHV-8 infection together with reactivation of Epstein- Barr-virus (EBV).

Conclusion

Primary CMV-infection and concomitant HHV-8 infection correlated with reactivation of EBV. We propose that these two viruses influenced the development and progression of the lymphoma. Quantitative PCR blood analysis for EBV, CMV and HHV-8 could be valuable in diagnosis and treatment of this type of very rapidly developing lymphoma. It is also a reminder of the importance of prevention and prophylaxis of several infections by having protected sex.
  相似文献   

4.
The safety and efficacy of reduced-intensity conditioning (RIC) followed by allogeneic stem cell transplantation (SCT) for relapsed lymphomas remains unresolved. We conducted a prospective, multicentered, phase II trial. A total of 170 relapsed/refractory lymphomas received a RIC regimen followed by SCT from sibling donors. The primary study end point was non-relapse mortality (NRM). Histologies were non-Hodgkin's lymphomas (NHL) (indolent (LG-NHL), n=63; aggressive (HG-NHL), n=61; mantle cell lymphoma (MCL), n=14) and Hodgkin's disease (HD, n=32). Median follow-up was 33 months (range, 12-82). The results show that frequencies were as follows: cumulative NRM at 3 years, 14%; acute and chronic graft-versus-host disease (GVHD) 35 and 52%, respectively; 3-year overall survival (OS), 69% for LG-NHL, 69% for HG-NHL, 45% for MCL and 32% for HD (P=0.058); and 3-year relapse incidence, 29, 31, 35 and 81%, respectively (P<0.001). Relapse risk differed significantly at 3 years between follicular lymphoma (FL) and chronic lymphocytic leukemia (CLL) (14 versus 46%, P=0.04). Molecular remission occurred in 94 and 40% (P=0.002) of patients with FL and CLL, respectively. On multivariate analysis, OS was influenced by chemorefractory disease (hazard ratio (HR)=3.6), diagnosis of HD (HR=3.5), and acute GVHD (HR=5.9). RIC allogeneic SCT is a feasible and effective salvage strategy in both indolent and aggressive NHL.  相似文献   

5.
Primary splenic lymphoma (PSL) is a rare malignancy representing about 1% of all lymphoproliferative disorders, when using a strict definition that allows only involvement of spleen and hilar lymph nodes. In contrast, secondary low-grade B-cell lymphomas in the spleen, such as follicular lymphomas (FL), lymphoplasmacytic lymphoma and chronic lymphocytic leukemia/ small lymphocytic lymphoma, particularly as part of advanced stage disease, are more common. Indolent B cell lymphomas expressing CD10 almost always represent FL, which in its primary splenic form is the focus of this review. Primary splenic follicular lymphoma (PSFL) is exceedingly infrequent. This type of lymphoproliferative disorder is understudied and, in most cases, clinically characterized by splenomegaly or cytopenias related to hypersplenism. The diagnosis requires correlation of histopathology of spleen, blood and/or bone marrow with the correct immunophenotype (determined by flow cytometry and/or immunohistochemistry) and if necessary, additional molecular profiling. Management of this incurable disease is evolving, and splenectomy remains the mainstream treatment for stage I PSFL.  相似文献   

6.
The majority of nasal lymphomas are of the natural killer (NK)/T cell lineage. We analyzed 33 specimens of nasal lymphoma from Japanese patients for Epstein-Barr virus (EBV). Phenotypic and genetic analyses showed 28 cases with NK/T cell type and 5 cases with B cell type. All NK/T lymphomas were of pleomorphic cell type except 2 large cell (centroblastoid) and one lymphoblastic lymphoma. All cases with nasal B cell lymphoma were of large (centroblastoid) cell type. EBV was detected in all cases of NK/T cell type with the exception of one lymphoblastic case, and was monoclonally integrated in all cases examined (14/14 cases). All but one case had subtype A of EBV infection with 30-base paired deleted LMP-1 gene. One case of B cell lymphoma showed the presence of EBV infection with subtype A and deletion of LMP-1. Our results indicate that the majority of nasal lymphomas in Japanese patients are of the nasal NK/T cell type, have pleomorphic morphology, a high prevalence of EBV with a monoclonal integration, subtype A and deleted LMP-1 gene. In contrast, nasal B cell lymphoma showed monomorphic appearance and rare EBV infection.  相似文献   

7.
S Falk  H J Stutte 《Cancer》1990,66(12):2612-2619
Seventeen cases of primary malignant lymphoma of the spleen (PMLS) were identified among 500 splenectomy specimens showing involvement by Hodgkin's disease or non-Hodgkin's lymphoma. All PMLS represented non-Hodgkin's lymphoma and most of them were of B-cell origin. In two cases PMLS were associated with hamartomas of the spleen (splenomas). Histologic and immunohistochemical studies did not reveal any differences between PMLS and disseminated malignant lymphomas with splenic involvement with regard to morphologic features, immunophenotype, host cell infiltrates, or proliferation activity. The reasons for the infrequent occurrence of primary lymphomas in the spleen may not be sought in a special immunophenotype of PMLS, a vigorous host response in the spleen, or in a lower proliferation activity of splenic lymphomas.  相似文献   

8.
The frequency of various subtypes of non-Hodgkin's lymphoma (NHL) differs in various regions worldwide. We studied distribution of various subtypes of NHL by using WHO classification of lymphoid neoplasms (2000), immunophenotyping and clinicopathologic characteristics of various histologic subtypes in 935 cases. B- and T-cell NHL constituted 79.3% and 18.8% of cases. Diffuse large B-cell lymphoma (DLBL) was the most common subtype (50.2%). A lower frequency of follicular lymphoma, marginal zone lymphoma and mantle cell lymphoma (MCL) was noted compared to that observed in the developed countries, whereas a lower frequency of peripheral T-cell lymphoma - not otherwise specified (PTCL-NOS) and extranodal NK/T-cell lymphoma was seen compared to that in the other Asian countries. A higher frequency of DLBL and precursor T-lymphoblastic leukemia/lymphoma was noted. Extranodal and bone marrow involvement in MCL and PTCL-NOS was less frequent. Anaplastic variant of DLBL was noted in 21.5% of all DLBLs. Null/T-cell anaplastic large cell lymphoma presented in the older age.  相似文献   

9.
CD30-positive anaplastic large cell lymphoma (ALCL) is a distinctive malignant large cell lymphoma of T-cell lineage, often presenting in lymph node or extranodal sites. ALCL cases with extensive bone marrow and peripheral blood involvement manifested as “leukemic phase” are extremely rare and the most of those cases reported are anaplastic large cell lymphoma kinase (ALK) positive ALCL in childhood population. Here we report four adult cases of ALK-negative ALCL with extensive bone marrow and peripheral blood involvement manifested as “leukemic phase”. Circulating large lymphoma cells varied from 20 to 80% in peripheral blood and bone marrow biopsy showed various nodular or interstitial infiltrates. By reviewing the clinicopathologic data of previously reported ALCL cases with extensive bone marrow and peripheral blood involvement, there appears to be of large variations in regard to the patient's age, morphologic variants, immunophenotypic or genotypic characteristics of the disease. While most cases of ALCL with peripheral blood and bone marrow involvement were ALK-positive or carrying t(2;5) translocation, rare ALK-negative cases were also present. Leukemic ALCL patients usually have unfavourable prognosis, regardless of ALK expression.  相似文献   

10.
De now CDS-positive (CD5+) diffuse large B-cell lymphoma (DLBL) has recently been identified as constituting a homogeneous subgroup with distinct clinicopathologic and genotypic characteristics, but its origin remains to he elucidated. Previous studies by sequence analysis of the variable region of the immunoglobulin heavy chain (VH) have shown that CDS5 B-cell malignancies such as mantle cell lymphoma (MCL) and B-cell chronic lymphocytic leukemia (B-CLL) cells represent pre-geminal center (pre-GC) stage B cells in contrast with the post-GC stage of most DLBLs, which show somatic hyper mutations in VH genes. In the present study, we investigated the VH sequence of de novo CD5+ DLBL to clarify whether CD5+ DLBL represents the pre-GC stage, as do other CD5+ B-cell malignancies, or the post-GC stage, as is typical of DLBL. All eight cases (four CDS4 DLBL and four CDS-negative (CD5) DLBL) examined by us showed somatic hypermutatiohs in the VH segment and two of the CD5 DLBL cases showed intra-clonal diversity, suggesting that CD5+ DLBLs were derived from the same maturation stage as CD5 DLBL, but were distinct from the other indolent CD5+ B-cell lymphomas of B-CLL and MCL. These data suggest that de novo CD5+ DLBLs do not merely lie within a continuous spectrum with B-CLL and MCL, but represent a biologically distinct variant within the diagnostic framework of diffuse large B-cell lymphoma.  相似文献   

11.
The aim of this retrospective study was to illustrate the clinicopathologic data and the treatment results in patients with primary gastrointestinal tract non-Hodgkin's lymphoma (GI NHL). Among 810 patients with NHL, 128 cases (15.8%) were diagnosed as primary GI tract NHL. There were 79 males and 49 females with median age of 62 years. The most common primary site was the stomach (68%). Overall, 67.2% of the patients were in stages I - II, and 32.8% in stages III - IV. Simultaneous involvement of the GI tract and other extranodal sites was observed in 26 patients (20%). Extranodal marginal zone B-cell lymphoma (MZBL) (i.e., low-grade lymphoma of mucosa-associated lymphoid tissue type) accounted for 48.4% of lymphomas. Aggressive lymphomas (diffuse large B-cell lymphoma [DLBL]) accounted for 44.5%. Eighty-three patients (67.5%) achieved complete response (CR), either by surgery (43/43 patients, 17 with DLBL and 25 with MZBL) or by primary chemotherapy (40/64 patients, 22 with DLBL and 17 with MZBL). Sixty-two patients remain in CR; 33/43 after surgical resection (13/17 with DLBL and 20/25 patients with MZBL), and 29/40 after only chemotherapy (18/22 with DLBL and 10/17 with MZBL). The major prognostic factor for outcome in the present study was the stage of the disease. Patients with localized lymphoma (stage I and II) had significantly longer DFS and OS (DFS and OS at 3-year: 83% and 87%, respectively) than patients with extended disease (stage III and IV) (DFS and OS at 3-year: 46% and 60%, respectively) (P < 0.0001). The International Prognostic Index (IPI) for patients with aggressive lymphomas was prognostic only for DFS (79% for low-risk patients [IPI score 0 - 1] vs 49% for higher risk groups [IPI score >1] at 3-year, P = 0.0131).  相似文献   

12.
Epstein–Barr virus (EBV) is a ubiquitous gammaherpesvirus that is associated with a variety of malignancies. In vivo infection of B lymphocytes is initially associated with the broad expression of immunodominant viral latency genes and proliferation of infected cells. Ultimately, a viral reservoir is established in resting B cells with restricted expression of viral latency genes and no expression of immunodominant viral genes. Among the tumours associated with EBV that are relevant to a consideration of EBV in HIV-associated malignancies are posttransplant lymphoproliferative disease, Burkitt's lymphoma (BL) and Hodgkin's disease (HD). BL carries whereas EBV in only a minority of cases whereas HD in patients infected with HIV is virtually always EBV-associated. EBV-directed T cell therapies have proven effective in posttransplant lymphomas in bone marrow transplantation patients. In patients with HIV infection, primary central nervous system (CNS) and immunoblastic lymphomas show similarities with posttransplant lymphoproliferative disease. EBV detection studies in cerebrospinal fluid are useful diagnostically in primary CNS lymphoma. T cell therapies may be useful in the treatment of EBV-associated lymphomas. Thus, a better understanding of the relationship between EBV and these tumours will not only help to clarify their pathogenesis, but may facilitate the development of new diagnostic and therapeutic strategies.  相似文献   

13.
不同部位和类型恶性淋巴瘤与EBV感染相关性研究   总被引:12,自引:0,他引:12  
目的:对不同部位和类型的恶性淋巴瘤中EB病毒(Epstein-Barr virus,EBV)的感染情况进行检测和分析,探讨EBV感染可能与恶性淋巴瘤的病因关系。方法:收集恶性淋巴瘤组织标本127例,其中鼻腔及鼻咽淋巴瘤60例,胃淋巴瘤30例,表浅淋巴结淋巴瘤37例。组织病理学诊断为非霍奇金淋巴瘤(NHL)108例,霍奇金淋巴瘤(HD)19例。采用原位分子杂交方法检测淋巴瘤组织中EBV编码的小RNA(EBER),确定EBV在恶性淋巴瘤细胞中的存在。结果:108例NHL(包括鼻腔及鼻咽、胃、表浅淋巴结部位),EBER检测46例阳性,阳性率为42.6%。鼻腔/鼻咽NHL的EBER阳性率为58.3%(35/60),其中NK/T细胞淋巴瘤29例,EBER阳性19例,阳性率为65.5%(19/29);B细胞淋巴瘤31例,EBER阳性16例,阳性率为51.6%(16/31)。胃部NHL的EBER阳性率为30.0%(9/30),其中B细胞淋巴瘤28例,EBER阳性9例,阳性率为32.1%(9/28);T细胞淋巴瘤2例,均为EBER阴性。表浅淋巴结NHL18例,EBER阳性2例,阳性率为11.1%(2/18);表浅淋巴结HD19例,EBER阳性5例,阳性率为26.3%(5/19)。结论:本组资料非霍奇金淋巴瘤EBV感染阳性率(42.6%)略高于霍奇金淋巴瘤EBV感染阳性率(26.3%),差异无显著性意义(P〉0.05)。鼻腔及鼻咽NHL的EBV感染阳性率(58.3%)高于胃NHL(30.0%)和表浅淋巴结NHL(11.1%)(P〈0.05)。研究提示各类型淋巴瘤与EBV感染均有密切关系,且存在部位依赖性。  相似文献   

14.
It is reported that overexpression of hnRNP A2 and B1 proteins is useful for detecting early cancers, and that B1, a splicing minor isoform of A2, is more specific than A2. The B1 expression is still undetermined in human lymphoid tissues. We quantitatively studied the B1 expression in 85 lymph node specimens, comprising reactive lymphoid hyperplasia (RLH; n=8), B-cell lymphoma (n=23), T-cell lymphoma (n=22), and metastatic carcinoma (n=32). Immunostaining and immunoblotting analyses with an anti-B1 monoclonal antibody, 2B2 were performed, and the two sets of results correlated with each other (p<0.05). In RLH specimens, B1 expression rate was significantly higher in follicular centers (FC; 44%) than in mantle zone (MZ; 15%) and paracortex (16%) (p<0.01). B1 expression was statistically higher in B-cell lymphoma than in T-cell lymphoma (p<0.01). In B-cell lymphomas, B1 expression rates were 51% in diffuse large B-cell lymphoma (DLBL; n=5) and 45% in follicular lymphoma (FL; n=16), and they were almost the same as that of the FC. Especially in DLBLs, CD10+ FC-origin lymphomas expressed greater amount of B1 than CD10- non-FC-origin lymphomas. B1 expression rate was low in mantle cell lymphoma (MCL; n=2) and similar to that of MZ in RLH. These results suggest that B1 expression is associated with differentiation in lymphoid tissue rather than transformation. B1 expression increases during the process of B-cell differentiation in the FC, and that high B1 expression is maintained in B-cell lymphomagenesis, especially in cells of FC-origin DLBL.  相似文献   

15.
Lymphomas occur with an increased frequency in patients with Human Immunodeficiency Virus (HIV) infection. These are usually high-grade immunoblastic lymphomas and primary central nervous system lymphomas. Anaplastic large cell lymphoma (ALCL) is a distinct type of non-Hodgkin's lymphoma. It is uncommon in HIV infected individuals. We describe here an uncommon presentation of this relatively rare lymphoma in the form of spinal cord compression syndrome in a young HIV infected individual.  相似文献   

16.
17.
CD30+ anaplastic large cell lymphoma (ALCL) represents a novel lymphoma entity at the borderline between Hodgkin's disease and non-Hodgkin's lymphomas. Phenotypic, genotypic, and karyotypic analyses have shown that ALCL are heterogeneous in cellular origin, and may be conceived as malignancies derived from activated, mainly T- or B-lymphoid cells, in some instances with an immature genotype. Epstein-Barr virus genomes and gene products, most notably the transformation-associated latent membrane protein (LMP), have been detected in a proportion of cases, and some cutaneous ALCL proved to harbor complete or incomplete HTLV-1 proviruses. These findings suggest that both EBV and HTLV-I, which are powerful inducers of CD30 expression in lymphoid cells in vitro, may contribute to the pathoetiology of ALCL.  相似文献   

18.
目的 探讨间变性大细胞淋巴瘤 (ALCL)的一种新类型—富于粒细胞的ALCL的临床病理特点。方法 收集 6例本院及会诊病例 ,免疫组化ABC法证实为ALCL ,使用抗体包括CD45,CD3 ,CD45RO,CD2 0 ,CD79,CD3 0 ,ALK1,EMA ,CD15,S -10 0 ,CD68,CD1a,均为DAKO产品。结果  6例均原发于淋巴结 ,其中 1例为B细胞性 ,5例为T细胞性 ,强表达CD3 0 ,不表达CD15,组织学特点为在肿瘤细胞间有大量的嗜中性粒细胞和 (或 )嗜酸性粒细胞浸润 ,局部形成小脓肿。结论 富于粒细胞的ALCL具有独特的组织学特点 ,可能是ALCL一种新亚型 ,但临床应与Langerhans细胞组织细胞增生症 (LCH) ,霍奇金淋巴瘤 (HL) ,T细胞淋巴瘤及急性化脓性炎症鉴别。  相似文献   

19.
20.

Background

B cell non Hodgkin lymphomas account for the majority of lymphomas in Uganda. The commonest is endemic Burkitt lymphoma, followed by diffuse large-B-cell lymphoma (DLBCL). There has been an increase in incidence of malignant lymphoma since the onset of the HIV/AIDS pandemic. However, the possible linkages of HHV8 and EBV to the condition of impaired immunity present in AIDS are still not yet very clearly understood.

Objectives

1. To describe the prevalence of Epstein-Barr virus, Human Herpes virus 8 and Human Immunodeficiency Virus-1 in B cell non Hodgkin lymphoma biopsy specimens in Kampala, Uganda. 2. To describe the histopathology of non Hodgkin lymphoma by HIV serology test result in Kampala, Uganda

Method

Tumour biopsies specimens from 119 patients with B cell non Hodgkin lymphoma were classified according to the WHO classification. Immunohistochemistry was used for detection of HHV8 and in situ hybridization with Epstein Barr virus encoded RNA (EBER) for EBV. Real time and nested PCR were used for the detection of HIV. The patients from whom the 1991-2000 NHL biopsies had been taken did not have HIV serology results therefore 145 patients biopsies where serology results were available were used to describe the association of HIV with non Hodgkin lymphoma type during 2008-2009.

Results

In this study, the majority (92%) of the Burkitt lymphomas and only 34.8% of the diffuse large B cell lymphomas were EBV positive. None of the precursor B lymphoblastic lymphomas or the mantle cell lymphomas showed EBV integration in the lymphoma cells. None of the Burkitt lymphoma biopsies had HIV by PCR. Of the 121 non Hodgkin B cell lymphoma patients with HIV test results, 19% had HIV. However, only 1(0.04%) case of Burkitt lymphoma had HIV. All the tumours were HHV8 negative.

Conclusions

The majority of the Burkitt lymphomas and two fifths of the diffuse large B cell lymphomas had EBV. All the tumours were HHV8 negative. Generally, the relationship of NHL and HIV was weaker than what has been reported from the developed countries. We discuss the role of these viruses in lymphomagenesis in light of current knowledge.  相似文献   

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