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  • 1
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Aims : To describe two cases of a peculiar pulmonary lesion, which expand both the morphological and the immunophenotypic spectrum of perivascular epithelioid cell (PEC)-related disorders.Methods and results : One man and one female, with and without the tuberous sclerosis complex (TSC), respectively, showed pulmonary cysts and small nodules on computed tomography scan. In the former, lymphangioleiomyomatosis (LAM) was suspected. In both cases, an open lung biopsy was performed, whose cut surface displayed numerous cysts lined by thin/thick septa. Microscopically, the septa were associated with micronodular or interstitial proliferation of medium/large-sized elements with abundant clear (periodic acid–Schiff-positive/diastase-sensitive) cytoplasm and distinct cell borders, embedded in fibrous tissue. The elements were CD34+, vimentin-positive and, to a lesser extent, HMB-45+ and MART-1+. The stains for specific muscle actin, desmin, S100 protein, CD31, FVIIIRAg, cytokeratins, CD45, CD68, oestrogen and progesterone receptors were all negative. Ki67 labelling was 〈1%. Electron microscopy displayed cytoplasmic vacuoles containing glycogen particles. The TSC1 and TSC2 gene status could not be assessed because of poor DNA preservation. In the man with TSC, a focus of micronodular pneumocyte hyperplasia was also found.Conclusions : Because of the coexpression of CD34 and melanoma-associated antigens and the occurrence of TSC in one patient, the cases described here add a new piece to the puzzle of PEC lesions and contribute to the open discussion on the origin of LAM and LAM-like proliferations.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Histopathology 44 (2004), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Aims:  To investigate CD27 expression in splenic marginal zone lymphoma (SMZL), an indolent low-grade B-cell lymphoma with constant involvement of the bone marrow, especially with an intrasinusoidal pattern. It is not clear if the neoplastic clone is composed of virgin or somatically mutated B cells. CD27 is reported to be a hallmark of memory B cells.Methods and results:  We evaluated 64 bone marrow biopsy specimens (BMBs) from 36 patients with SMZL for the expression of CD27. For comparison, splenectomy specimens of patients with traumatic splenic rupture or with SMZL were used. All BMBs showed lymphomatous infiltration. When located in the marrow sinusoids, neoplastic cells were CD27– in all cases and therefore corresponded to naive B cells. In nodular/interstitial infiltration, the cells were CD27+ and therefore corresponded to memory B cells. No difference in immunohistochemical expression of B and T antibodies was found between intrasinusoidal and interstitial/nodular infiltration. CD27 was constantly expressed in the splenic marginal zone of normal spleen, surgically removed for trauma, and in seven out of 10 spleens with SMZL.Conclusion:  We propose the existence of two different phases of neoplastic progression with, first, expansion of a virgin B clone in the bone marrow and, following exposure to antigen, a re-colonization of the bone marrow.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, U.K. and Cambridge, USA : Blackwell Science Ltd
    Histopathology 30 (1997), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Diffuse large B-cell lymphoma (DLBCL) is the commonest type of lymphoid tumour world-wide. This category was included both in the REAL and WHO Classification aiming to lump together all malignant lymphomas characterized by the large size of the neoplastic cells, B-cell derivation, aggressive clinical presentation, and the need for highly effective chemotherapy regimens. These tumours are detected as primary or secondary forms both at the nodal and extranodal levels, in immunocompetent hosts as well as in patients with different types of immunosuppression. They display a significant variability in terms of cell morphology and clinical findings, which justifies the identification of variants and subtypes. Among the latter, the primary mediastinal one does actually correspond to a distinct clinicopathological entity. Immunophenotypic, tissue microarray and molecular studies underline the extreme heterogeneity of DLBCLs and suggest a subclassification of the tumour, based on the identification of different pathogenic pathways, which might have much greater relevance than pure morphology for precise prognostic previsions and adoption of ad hoc therapies. The more recent acquisitions on the pathobiology of DLBCLs are reviewed in the light of the authors' experience, aiming to contribute to the existing debate on the topic.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1569-8041
    Keywords: Hodgkin's disease ; immunohistochemistry ; in situ hybridization ; liver transplantation ; post-transplant lymphoproliferative disorder
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Here we describe the case of a 14-year-old boy who underwent livertransplantation for post-Kasai biliary atresia when aged 4. Antirejectiontreatment consisted of prednisone and cyclosporine. At the age of 11 years thepatient developed left cervical lymphadenopathy; the biopsy showed classicalHodgkin's disease(HD) of the mixed cellularity (MC) type. Neoplastic cellsexpressed CD30 and CD15, and were negative for CD45, CD20, CD3, CD43, andCD79a. Furthermore, they carried the EBV-related products LMP1 and EBER1/2.Treatment consisted of three cycles of adriamycin, bleomycin, vinblastine andDTIC (ABVD), followed by radiotherapy (2,000 cGys) on involved fields. Atpresent, 42 months after the diagnosis of HD, the patient is still in completeremission. This is, to the best of our knowledge, the first reported case ofclassical HD following liver transplantation. The positivity of neoplasticcells for LMP1 and EBER1/2 indicates a possible role for immunosuppression inthe development of the tumor, and whether a reduction in immunosuppressionmight have influenced the course of the disease is open to question.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Annals of oncology 9 (1998), S. 797-801 
    ISSN: 1569-8041
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1569-8041
    Keywords: cytogenetics ; peripheral T-cell lymphoma ; phenotype ; T-cell chronic lymphocytic leukaemia ; T-cell prolymphocytic leukaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract T-cell chronic lymphocytic leukaemia (T-CLL) has recently been reclassified under the heading of T-cell prolymphocytic leukaemia (T-PLL) because of its unfavourable clinical course, independently of the morphologic features. This rare neoplasm usually shows CD4+/CD8− phenotype. Herein we report on two cases of T-PLL with CD8 expression that correspond to a possible variant of the disease first proposed by Hui et al. in 1987. These cases presented with malignant cells showing immunophenotypic features that can be easily identified and distinguished from other peripheral T-cell leukemias. However, the total number of cases studied is inadequate for defining a discrete clinico-pathologic entity with characteristic clinical features and cytogenetical abnormalities. An international collaboration in which tissue from similar cases is referred to a central pathologist for immunophenotyping and cytogenetical study, and clinical data are centrally compiled, may assist in defining this rare malady as a discrete clinico-pathologic entity.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1569-8041
    Keywords: EBV ; immunohistochemistry ; malignant lymphoma ; molecular biology ; pleuritis ; pneumothorax ; pyothorax ; tuberculosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background: Pyothorax-associated lymphoma (PAL) is a rare, but distinct, clinico-pathologic entity which occurs most often in Japanese people; to the best of our knowledge, only six cases of it have been reported in Western countries. The tumour develops several decades following artificial pneumothorax or chronic pleuritis due to tuberculous infection, produces pleural effusion associated with extensive local lymphomatous infiltrates, and is sustained by a polymorphic large B-cell clonal proliferation showing EBV integration in the genoma of the neoplastic cells. Patients and methods: Herein we describe two cases of PAL observed in Italian patients, both extensively studied on the clinical, pathological, phenotypic, virological, and molecular levels. Results: The two cases occurred, respectively, 45 and 50 years after therapeutic pneumothorax because of tuberculous pleuritis and were characterized by a pleural mass extending to the thoracic wall, which on histological examination were seen to consist of large elements with immunoblastic morphology. Immunohistochemistry showed monotypic restriction of Ig light chains, as well as the expression of CD45, B-cell markers (CD20, CD79a, CD45RA), bcl-2 oncogene product, EBNA-2 and, partially, LMP-1. The ratio of cycling cells was extremely high as was the number of mitotic figures. In situ hybridization displayed the presence in the neoplastic cells of the EBV-related small RNAs EBER 1 and 2, which in turn, along with the positivity for EBNA-2 and LMP-1, further strengthened the close relationships between PAL and latent viral infection. Molecular studies revealed, on one hand, clonal rearrangement of the Ig heavy chain J region genes, and on the other, negativity for HHV8 in one case and positivity in the other. Conclusions: These cases of PAL are the first to be documented in Italy; they serve to direct attention to the fact that this condition is not confined to Japanese people, and that its occurrence in Western countries might be underestimated.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1569-8041
    Keywords: α-interferon ; low-grade MALT lymphoma ; nongastrointestinal ; radiotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background: Peripheral B-cell lymphoma of the marginal zone (MALT, low-grade), presenting as localized, extranodal disease, usually affects the elderly. The gastrointestinal tract is the most frequently involved extranodal location, representing 70% of all MALT lymphomas. Recently, numerous other extranodal sites involved by MALT lymphomas have also been described. Patients and methods: From January 1990 to October 1995, 24 patients with untreated nongastrointestinal low-grade MALT lymphoma were submitted to treatments ranging from the local approach of radiotherapy and local α-interferon (α-IFN) administration to chemotherapy. The tumours were located in the lung (seven cases), conjunctiva (four cases), lachrymal gland and orbital soft tissue (four cases), salivary glands (three cases), skin (three cases), breast (two cases)' and thyroid (one case). All patients had low-grade stage IE tumours. Results: Chemotherapy was administered in 11 patients (six with lung, three with salivary gland, one with breast, and one with thyroid locations); radiation therapy was employed in seven patients (three with lachrymal gland, three with skin, and one with breast locations); local α-IFN administration was administered in five patients (four with conjunctival, and one with lachrymal gland sites); and surgery was employed in one patient with a lung tumour. All patients achieved complete remissions; three local recurrences and two relapses in other sites were observed. The global five-year survival rate was 100% with a relapse-free survival rate of 79%. Conclusions: These data confirm the significant efficacy of different therapeutic approaches to specific sites inbes obtaining a good remission rate for nongastrointestinal localized low-grade MALT lymphomas.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1569-8041
    Keywords: anaplastic large cell lymphoma ; classification ; clinics ; histology ; immunohistochemistry ; peripheral T-cell lymphoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background: One hundred sixty-eight peripheral T-cell lymphomas (PTCLs)were reviewed according to the Revised European–American Lymphoma (R.E.A.L.)Classification. Patients and methods: The cases, originally diagnosed on the basis of theUpdated Kiel Classification (UKC), were all provided with histologicalpreparations, immunophenotype, clinical information, and follow-up data. Theslides were reclassified by five observers, who integrated the R.E.A.Lcriteria with cell size measurements. The prognostic value of clinical andpathologic findings was assessed by univariate and multivariate analysis. Results: The R.E.A.L. Classification was reproducibly applied by all of theobservers. Clinically, anaplastic large cell lymphomas (ALCLs) differed fromthe remaining PTCLs by mean age (29.5 vs. 52.9 years), bulky disease(52.3% vs. 11.3%; P = 0.000), mediastinal mass (52.7% vs.32%; P = 0.004), and disease-free survival (68.0% vs.38.2%; P = 0.0001). Although each histological type displayed specificclinical aspects, PTCLs other than ALCL were basically characterised by a poorclinical outcome which was not influenced by the UKC malignancy grade. Atmultivariate analysis, the risk of a lower complete remission rate was relatedto bulky disease (P = 0.001), histologic group (non-ALCL) (P = 0.01), andadvanced stage (III–IV) (P = 0.0002). Conclusions: The present study supports the classification of T-celllymphomas proposed by the R.E.A.L. scheme.
    Type of Medium: Electronic Resource
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