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Anaplastic large cell lymphoma (ALCL), CD30+, is a subtype of T-non-Hodgkin's lymphoma (NHL). Its most common form is a classical systemic type that involves multiple nodal and extranodal sites. In this study, morphologic, immunohistologic, and genetic studies were performed on ALCL cases in Pakistani patients. The median age of the patients in this study was 45 years (age range: 5-70 years), with a male to female ratio of 3.4:1. Thirty-seven (37) patients were diagnosed to have Ki-1 (CD30+) ALCL, which constituted 2% of all NHLs and 12.6% of all T-NHLs, over a period of 11 years (January 01, 1992-December 31, 2002). The tumors were of either T- or null-cell type with constant (100%) expression of CD30 (Ki-1). The majority of the cases (89.2%) expressed EMA, whereas 40.5% of the cases expressed either CD45 (LCA), CD45RO (UCHL1), or ALK. The mean age of ALCL patients with null-cell phenotype was 33.8 years as compared to those with T-cell phenotype having a mean age of 36.3 years. Out of the 37 cases diagnosed as ALCL, amplifiable DNA was isolated from 28 cases, which were further assessed for T-cell clonality for T-cell receptor (TCR)-beta, gamma, and immunoglobulin heavy chain (IgH) for the FR2 and FR3 regions. The polymerase chain reaction (PCR) technique demonstrated clonal rearrangement of the TCR beta, gamma, and IgH regions in 15 (53.6%), 11 (39.3%), and 2 (7.1%) ALCL cases, respectively, out of 28 cases. Association of Epstein-Barr virus (EBV) was noted in seven out of 28 cases (25%) of ALCL by PCR, whereas ISH for EBV-encoded nuclear RNA-1 (EBER-1) detected the presence of EBV in two (16.7%) out of 12 cases, where one was T-cell ALCL and the other null-cell ALCL. Immunostaining for LMP-1 could not be performed, because tissue material was not available. In conclusion, our study demonstrated that the prevalence of ALCL in Pakistan is comparable to that reported for some of the Asian communities and by the International Lymphoma Study Group and that EBV could be partly responsible for the pathogenesis of ALCL.
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PMID:Prevalence and characterization of anaplastic large cell lymphoma and its association with Epstein-Barr virus in Pakistani patients. 1564 4

Histological, clinical and immunohistochemical analysis of 6 cases of primary liver lymphomas (PLL) are presented. PLL represents 4.3% of primary malignant liver tumors diagnosed in our department. The patients were relatively young people, who despite the presence of a large tumor, were in good general health status. There were no signs of scirrhosis, and cancer markers were normal. All lymphomas were CD20, CD79a, BAX positive, CD3, CD30, EMA, CD10, CD5, CD59, c-myc, Bcl2, EBV(LMP), CK negative. The proliferation index (Ki67) was high, ranging from 50-100%. In two cases positive staining for Bcl6 and in another one for cyclin D1 was obtained. The major histological type of the tumor was diffuse large B-cell lymphoma. Positive immunohistochemical results with BAX and the lack of Bcl2, c-myc and CD59 are associated with better prognosis. We have not confirmed the value of Bcl6 and CD10 stains as a predictor of poor outcome. Despite clinically advanced stage at the time of diagnosis, if treated appropriately, the primary lymphoma of the liver has relatively good prognosis (five of our patients are alive).
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PMID:Primary lymphoma of the liver -- morphological and clinical analysis of 6 cases. Success of aggressive treatment. 1587 91

Plasmablastic lymphoma is an HIV-associated non-Hodgkin's lymphoma that primarily affects the oral cavity and jaws. The purpose of this report is to describe the first case of plasmablastic lymphoma occurring in an HIV-negative, nonimmunocompromised individual, and to review the histopathologic and immunohistochemical phenotype of this lymphoma. Histopathologically, our case exhibited a dense, diffuse lymphocytic infiltrate of noncohesive large lymphocytes with plasmacytoid features. Immunohistochemical analysis revealed positivity for the B-cell marker CD79a, VS38c, Epstein-Barr virus latent membrane protein (LMP), immunoglobulin G (IgG), and lambda light chain restriction. Neoplastic cells were negative for leukocyte common antigen, CD20, CD3, CD10, CD138, Bcl-2, Bcl-6, desmin, actin, EMA, S-100, HMB45, Alk-1, HHV8, IgA, IgM, and cytokeratin. The features of this rare disease are summarized based on a comprehensive review of the epidemiologic, clinical and immunohistochemical findings of previously reported cases.
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PMID:Oral plasmablastic lymphoma in an HIV-negative patient: a case report and review of the literature. 1603 78

PEL, a rare type of lymphoma constituting less than 5% of NHLs, has been recently identified as a distinct clinical and pathological entity among the B-cell lymphomas, with characteristic morphologic, immunophenotypic, molecular and viral features. ICC, PCR, RT-PCR and sequencing were carried out in biologicals samples from a 44-year-old, non-smoker Caucasian male patient of Greek nationality, HIV-1 negative and HCV positive. The ICC results showed CD30 + , Vimentin + , EMA + , Ki67 + , Pankeratin- and negative to B and T antibodies. In addition, HHV-8 was detected in pleural fluid. Examination of blood samples of the patient over a period of nearly two years showed a persistent infection of HHV-8. Phylogenetic analysis revealed a close relation to the C1 variant of HHV-8. The samples was also found EBV negative by PCR. Using a combination of clinical, morphological, immunohistochemical features and molecular biology techniques in this study we document a PEL case with persistent HHV-8 of genotype C1 infection.
Leuk Lymphoma 2005 Oct
PMID:Subtype C1 persistent infection of HHV-8 in a PEL patient. 1619 97

This study aims to assess the distribution of lymphoma subtypes in Shanxi, China, according to the World Health Organization (WHO) classification, and to compare the relative distribution with other areas of the world. H&E-stained tissue sections from the archives of the Shanxi Tumor Hospital, China, were reviewed and 447 cases with sufficient materials were selected for detailed study. A panel of antibodies and probes was assembled, including antibodies to ALK1, bcl-6, CDs 1alpha, 3, 4, 5, 7, 8, 10, 15, 20, 23, 30, 43, 56, 68, 79alpha, and 99, cyclin D1, EMA, kappa, lambda, LMP1, PAX5, TdT, Vs38C and ZAP70, plus EBER RNA probe by in situ hybridization. The 447 lymphoma cases, subtyped according to the WHO classification, were assembled in triplicate into 11 tissue microarrays and examined with the panel of markers described. Among the 447 cases, 385 (82.6%) were confirmed to be non-Hodgkin lymphomas (NHL) and 62 (13.9%) were Hodgkin lymphomas of classic type (CHL). Of the NHL cases, 68.6% were B-cell lymphomas and 30.6% T/NK-cell lymphomas. Histiocytic neoplasms accounted for only three cases (0.8%). Diffuse large B-cell lymphomas (DLBCL) were the most common subtype (35.1%), followed by peripheral T-cell lymphomas unspecified (PTun, 12.0%), extranodal marginal zone B-cell lymphomas (MALT lymphomas, 11.7%), follicular lymphomas (FL, 8.6%), T-lymphoblastic lymphomas (T-LBL, 7.0%), anaplastic large cell lymphomas (ALCL, 4.2%), B small lymphocytic lymphomas (B SLL, 3.6%), and mantle cell lymphomas (MCL, 2.6%). Of 263 B-cell neoplasms, 105 (39.9%) expressed immunoglobulin light chain, including 52 kappa and 53 lambda, detectable in paraffin sections. The incidence of DLBCL was similar to many Western countries and Asia. The frequency of FL was, however, much lower than the usual pattern in Western countries, although NK/T-cell lymphomas were more common (30.6%), similar to other countries in Asia, including Japan and Korea. With regard to markers of EBV infection, 8 of 385 (2.1%) NHL cases gave positive findings by both in situ hybridization (EBER RNA) and immunohistochemistry (LMP-1), whereas 24 (6.2%) expressed only the EBER and 12 (3.1%) expressed only LMP-1. EBV positivity was found in 24 of 119 (20.2%) T and NK cell lymphomas, in 20 of 263 (7.6%) B cell neoplasms, and in 37 of 62 (59.7%) CHLs. In CHLs there was complete concordance of results by both in situ hybridization (EBER RNA) and immunohistochemistry (LMP-1) procedures. ZAP70 was detected in most T cell-lineage disorders (61.4%) and also in a subset of B small lymphocytic lymphomas (50%). However, ZAP-70 was expressed in a minority of other types of B-cell lymphomas, including precursor B-cell acute lymphoblastic leukemia (25%), diffuse large B-cell lymphoma (26.7%), follicular lymphoma (15.2%), and lymphoplasmacytic lymphoma (9.1%). Immunohistochemical analysis represents an effective method for assessing ZAP-70 expression and reveals that a variety of B-cell malignant neoplasms express ZAP-70, albeit at low frequency.
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PMID:Distribution and ZAP-70 expression of WHO lymphoma categories in Shanxi, China: a review of 447 cases using a tissue microarray technique. 1628 Jun 61

We report the case of a 77-year-old Japanese man with natural killer (NK)-like T cell lymphoma of the small intestine diagnosed after an emergency laparotomy for perforated peritonitis. Immunohistochemical staining of the tumor showed that the patient had CD3+ CD8+ CD30- CD56+ CD68- CD79a- UCHL-1+ EMA- LMP-1 NK-like T cell lymphoma. The patient had a history of hepatocellular carcinoma (HCC) and was also diagnosed with T cell non-Hodgkin's lymphoma associated with T cell receptor (TCR) reconstruction in the Jgamma chain. Intestinal T cell lymphoma is uncommon and very few cases of CD56+ T cell lymphoma, otherwise known as NK-like T cell lymphoma, have been reported. The patient did not have a history of gluten-sensitive enteropathy (celiac disease). Multiple lesions appeared within months after the initial operation and his condition deteriorated rapidly. We think that this patient probably had NK-type granular lymphocyte-proliferative disorder (NK-GLPD) because the percentage of CD16+ CD56+ cells among peripheral blood mononuclear cells was elevated, at 21%. We report this case to help elucidate the relationship between underlying digestive organ disease and the development of intestinal NK-like T cell lymphoma. An accumulation of other such cases is needed to determine the etiology of this disease.
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PMID:Natural killer-like T cell lymphoma of the small intestine: report of a case. 1663 56

A 40-year-old previously healthy lady presented with nasal obstruction and localized plaques over the right arm. She developed complete nasal obstruction due to a mass in the right nasal cavity and skin lesions that ulcerated to present as ecthyma gangrenosum like lesions. Patient's condition deteriorated fast and she developed icterus with fatal outcome within 4 weeks of developing skin lesions. Nasal and skin biopsy revealed angiocentric T-cell lymphoma, which on immuno-phenotyping revealed CD-3 positive; and CD-20, CD-30, ALK and EMA negativity. She was seronegative for HIV. Final diagnosis of CD-3 positive extranodal T-cell lymphoma of nasal type was made. Extranodal T-cell lymphomas are very aggressive NHLs with poor prognosis. Prognosis depends on histology, stage of the disease and sites of involvement. NK/T cell lymphoma of nasal type is common with EBV association. Skin involvement is rare and is also an indicator of poor prognosis.
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PMID:CD-3 positive extranodal T-cell lymphoma of nasal type with skin involvement. 1676 37

The recent WHO classification of non-Hodgkin's lymphoma is based on the morphology and immunohistochemical expression of the lymphoma cells and to a lesser extent, on the molecular and cytogenetic findings. Fifty-three cases of non-Hodgkin's lymphoma were included in the study. Of these, seven cases were primary extra nodal lymphomas. Twenty two patients had peripheral blood and/or bone marrow involvement. A detailed morphological assessment was done and classified using the International working formulation. The two most common types encountered were diffuse large cell lymphoma and small lymphocytic lymphoma. Immunohistochemistry was done using labeled streptavidin-biotin peroxidase complex with CD3, CD20, CD15, CD30, CD 45 (leukocyte common antigen), Cyclin D1, EMA (epithelial membrane antigen). 38 cases (72%) showed B cell expression and 12 cases (22.5%) showed T cell expression. Three cases did not express either marker. B-cell diffuse large cell lymphoma (26%) was found to be the predominant B cell non-Hodgkin's lymphoma. The commonest T-cell lymphoma was T lymphoblastic lymphoma (67%) followed by peripheral T cell angioimmunoblastic lymphoma (25%). Immunohistochemistry is a useful and necessary diagnostic modality and helps subdivide prognostically different types of non-Hodgkin's lymphoma.
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PMID:Immunohistochemical typing of non-Hodgkin's lymphoma-comparing working formulation and WHO classification. 1693 15

We describe an unusual case of ALK-1-positive primary mediastinal lymphoma with the morphology of an anaplastic large-cell lymphoma (ALCL) of T/NK cell type but expressing CD20. This tumour had T/NK morphology and immunophenotype, as demonstrated by its expression of CD30, EMA, ALK-1, CD7 and TiA-1 and the lack of expression of B-cell markers other than CD20. The significance of such a co-expression of a B cell-associated antigen in a case of ALCL of T/NK cell type is discussed.
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PMID:Primary mediastinal anaplastic alk-1-positive large-cell lymphoma of T/NK-cell type expressing CD20. 1725 28

Non-Hodgkin lymphomas of the breast are rare, encompassing approximately 0.04-0.5% of all malignant breast tumors, and the vast majority are B-cell lymphomas. In contrast, lymphomas of T-cell phenotype have been rarely reported and some of these have been in close proximity to a breast implant. In our consultation practice, we have identified four patients with primary T-cell anaplastic large-cell lymphoma presenting adjacent to silicone or saline breast implants. All patients presented with seroma and neoplastic cells were identified in suspension in the serous fluid without solid tissue invasion. Three patients had no evidence of systemic disease (stage 1E), and one patient was not staged. The mean age of the patients was 46 years (range, 34-59 years). In all patients, the neoplastic cells had a T-cell phenotype, expressed CD30, cytotoxic granule-associated proteins, EMA and clusterin, and were anaplastic lymphoma kinase-1-negative. Clonal T-cell receptor gamma-chain gene rearrangements were identified in three patients. All patients underwent capsulectomy with removal of the implant. One patient subsequently received chemotherapy and radiation therapy, and another was treated with radiation alone. The third patient received no further therapy and the fourth patient has been recently diagnosed. After a mean time of 13 months (range, 9-20 months), all three patients with follow-up were alive and well without any recurrence or systemic disease. Although the follow-up time was relatively short, our series and other reported cases suggest that primary anaplastic large-cell lymphoma adjacent to breast implants is an indolent T-cell lymphoproliferative disorder.
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PMID:Seroma-associated primary anaplastic large-cell lymphoma adjacent to breast implants: an indolent T-cell lymphoproliferative disorder. 1822 53


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