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  • 1
    Abstract: We recently reported a truncating deletion in the NFKBIE gene, which encodes IkappaB, a negative feedback regulator of NF-kappaB, in clinically aggressive chronic lymphocytic leukemia (CLL). Preliminary data indicate enrichment of NFKBIE aberrations in other lymphoid malignancies, hence we screened a large patient cohort (n=1460) diagnosed with different lymphoid neoplasms. While NFKBIE deletions were infrequent in follicular lymphoma, splenic marginal-zone lymphoma, and T-cell acute lymphoblastic leukemia (〈2%), slightly higher frequencies were seen in diffuse large B-cell lymphoma, mantle cell lymphoma, and primary CNS lymphoma (3-4%). In contrast, a remarkably high frequency of NFKBIE aberrations (46/203 cases, 22.7%) was observed in primary mediastinal B-cell lymphoma (PMBL) and Hodgkin lymphoma (3/11 cases, 27.3%). NFKBIE-deleted PMBL patients were more often therapy-refractory (P=.022) and displayed inferior outcome compared to wildtype patients (5-year survival: 59% vs. 78%; P=.034); however they appeared to benefit from radiotherapy (P=.022) and rituximab-containing regimens (P=.074). NFKBIE aberrations remained an independent factor in multivariate analysis (P=.003), also when restricting to immunochemotherapy-treated patients (P=.008). Whole-exome sequencing and gene expression-profiling verified the importance of NF-kappaB deregulation in PMBL. In summary, we identify NFKBIE aberrations as a common genetic event across B-cell malignancies and highlight NFKBIE deletions as a novel poor-prognostic marker in PMBL.
    Type of Publication: Journal article published
    PubMed ID: 27670424
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  • 2
    ISSN: 1432-1076
    Keywords: Cushing syndrome ; Primary adrenocortical nodular dysplasia ; Adrenal stimulating immunoglobulins ; Autoimmune involvement ; Familial occurrence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cushing syndrome due to primary adrenocortical nodular dysplasia was diagnosed in two patients, aged 3 years 9 months and 9.5 years. Subsequently, adrenalectomy was performed and followed by steroid replacement. In both cases, the adrenals were normal or only slightly enlarged and showed adrenocortical nodular dysplasia histologically. Small lymphocytic infiltrates consisting of T-cells and class II MHC positive macrophages were present in adrenal specimens of both the patients. Samples of protein A sepharose purified serum immunoglobulins from both children stimulated adrenocortical DNA synthesis and cortisol production in cultured guinea-pig adrenal segments in vitro in a dose dependent fashion. Adrenal stimulating immunoglobulins were also demonstrated in serum specimens of both patients' mothers. However, none of them had overt signs of adrenal disease. Our data support the view that autoimmune mechanisms may be involved in primary adrenocortical nodular dysplasia.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-2307
    Keywords: Langerhans cell histiocytosis ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The immunophenotypic properties of the abnormal cells in routine specimens from 16 cases of Langerhans cell histiocytosis (LCH) were examined. In five cases, cryostat sections were also available. The abnormal cells expressed a similar phenotype and were positive for HLA-DR, S-100 protein, peanut agglutinin (PNA), CD1a, CD4 and several macrophage-associated markers, including CD11c, CDw32 and CD68 (the latter detectable in routine sections with antibody KP1). Staining with CD14, CD35 (C3b receptor), and CD11b (C3bi receptor) was negative with the exception of one of the cases in which a proportion of the cells showed faint positivity with CD11b. Staining for pan-T-cell (CD2, CD3, CD5) and panB-cell (CD 19, CD22) antigens was negative in all lesions. It is concluded that LCH expresses a characteristic phenotype with some heterogeneity with regard to macrophage markers and that immunohistochemical methods in cryostat sections and routine specimens form a useful supplement to other techniques for the diagnosis of this condition.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0584
    Keywords: Key words Splenic rupture ; T-cell lymphoma ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Pathological or spontaneous rupture of the spleen has been described in a variety of diseases affecting the spleen, with infections being cited as the cause in most cases. In hematological malignancies it is a rare event, despite the frequent involvement of the spleen in these diseases. It has, however, been described in patients with acute and chronic leukemia, Hodgkin's disease, non-Hodgkin's lymphoma of B-cell origin, mycosis fungoides, and so-called histiocytic lymphoma. Here, we present a fatal case of splenic rupture caused by infiltration of a peripheral T-cell lymphoma, unspecified according to the REAL classification. The importance of a correct diagnosis and fast surgery is emphasized.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-069X
    Keywords: Dermal lymphocytic infiltrates ; Psoriasis ; Flow cytometry ; DNA measurements
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Single-cell DNA measurements obtained using flow cytometry have previously been used as a diagnostic tool for various malignant diseases. We used this technique to characterize the dermal infiltrates of 14 patients with psoriatic skin lesions and 22 patients with various benign skin disorders. The DNA histograms of all of the patients exhibited an increased number of cells showing propidium-iodide fluorescence in the hyperdiploid region as compared to those seen in control DNA histograms of normal blood mononuclear cells. The calculated DNA indices revealed one hyperdiploid (G0/G1) peak in 17 cases and two hyperdiploid (G0/G1) peaks in 13 cases. The results suggest that the dermal lymphocytic infiltrate consists of (1) lymphocytes in the cell-division cycle, (2) non-cycling lymphocytes (G0) and (3) activated lymphocytes that may either remain in a non-cycling state or enter the cell cycle.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The presence of intercellular adhesion molecule-1 (ICAM-1) on keratinocytes of psoriatic skin lesions before and during 8-methoxapsoralen and UVA light (PUVA) treatment was studied. ICAM-1 was expressed on the keratinocytes in biopsies of the skin lesions of five patients with psoriasis. The patients who responded to PUVA treatment had a concurrent reduction of ICAM-1 expression on the keratinocytes with a reduction of the number of cells in the mononuclear cellular infiltrate and a lessening of the severity of the disease. Patients who went into remission during therapy and then relapsed showed an increase in ICAM-1 expression on keratinocytes with an increase in the number of cells in the mononuclear cell infiltrate and an increase in the severity of the disease. HLA-DR expression on keratinocytes was variable during treatment and showed no strong correlation with disease severity.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The clinical, histological, phenotypic and genotypic features of 21 primary cutaneous B-cell lymphomas (CBCLs) have been investigated. The patients were 13 men and eight women aged 34–91 years (median 67) at diagnosis. Eighteen patients had localized disease, and three had multiple skin lesions at diagnosis. Twelve patients developed cutaneous or extracutaneous recurrences, and five died from malignant lymphoma 7–84 months (median 36) after diagnosis. Histological examination showed features of marginal zone/mucosa-associated lymphoid tissue (MALT)-type lymphoma in 12 cases. Three of these had transformed to diffuse large B-cell lymphoma (DLBCL) in relapse biopsies. The remaining cases were seven primary DLBCLs and two cases tentatively classified as follicle centre cell (FCC) lymphoma. The neoplastic B cells showed similar phenotypes and genotypes in most cases (CD20+, CD79+, CD5–, CD10–, cyclin D1–, bcl-2+, bcl-x–, bax–, t(14;18)-negative). p53 protein was expressed in five cases, and four harboured mis-sense or loss-of-function mutations in the p53 gene. Deletion or promoter region hypermethylation of the p16INK4a gene was detected in two patients with DLBCL. The level of retinoblastoma protein expression and the proliferative fraction were significantly higher in DLBCL (〉 50%) than in MALT- or FCC-type lymphomas (〈 10%). Features associated with an unfavourable prognosis were the presence of multiple skin lesions at diagnosis, transformation from MALT-type lymphoma to DLBCL, and possibly p16INK4a aberrations. It is concluded that most CBCLs are dissimilar from FCC lymphomas and seem to be more closely related to marginal zone/MALT-type lymphomas. It is also suggested that there are fundamental differences between DLBCL and other histological categories of CBCL, indicating that cutaneous DLBCL is a separate entity with an increased growth potential and genetic features similar to DLBCL originating in other anatomical sites.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 133 (1995), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: p53 is an oncosuppressor gene located on chromosome 17p. Point mutations of the p53 gene are seen frequently in human malignancies, and are closely associated with malignant transformation under in vitro conditions. Mutated p53 protein shows a slow cell turnover rate, and accumulates in cells at the nuclear and/or cytoplasmic level. As a result, mutated p53 protein can be detected more readily by immunohistology than the wild-type protein. In this study, we used a monoclonal anti-p53 antibody (clone D07) to examine the expression of p53 protein in 25 cutaneous T-cell lymphomas (CTCL) of low- and high-grade malignancy, i.e. mycosis fungoides (n = 6), Sézary's syndrome (n = 2), and large cell lymphomas of pleomorphic (n = 14) or anaplastic (n= 3) subtype. The results showed that easily detectable p53 protein was present in many of the neoplastic cells in half of the high-grade lymphomas. In contrast, in the low-grade lymphomas no, or only very few, p53-positive neoplastic cells could be detected. These findings suggest that molecular and/or genetic alterations of p53 may be implicated in the pathogenesis of high-grade CTCL, but are unlikely to be of critical importance in low-grade CTCL.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    ISSN: 1365-2230
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Single cell DNA measurements have recently been used in diagnosis and as a measure of treatment response in different premalignant and malignant conditions. In the present case evolution of cutaneous lymphoid hyperplasia to cutaneous T-cell lymphoma was demonstrated by histological and immunohistological criteria. However, using single cell DNA measurements clear aneuploidy, by an additional peak in the hyperdiploid region of the DNA histogram, was revealed both in the benign and malignant stages of the disease. Lymph node and peripheral blood were normal by histopathological, immunohistopathological and single cell DNA measurements, indicating that the malignant potential may have been confined to the skin. Thus, single cell DNA measurements may be used as an additional diagnostic tool, when the diagnosis is inconclusive or at variance with the clinical findings.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1365-2230
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: In this study we have investigated the configuration of the T–cell receptor (TCR) β–chain genes in benign cutaneous conditions (n= 5) and known (n= 22) or suspected (n= 5) cutaneous T–cell lymphoma (CTCL). Sequential biopsies from skin, lymph node, blood and/or bone marrow were available in 12 cases of the 22 confirmed CTCL, and a total of 67 samples were analysed. In the benign conditions, clonal rearrangements of the TCR β–chain genes were seen in neither skin nor blood samples. In contrast, in CTCL clonal rearrangements were detected in all skin samples from plaque or tumour lesions of mycosis fungoides. Clonal TCR rearrangements were also present in skin and blood samples from two patients with Sèzary's syndrome, and in skin and blood samples from three of five patients with clinically suspected CTCL. In 10 patients with large cell lymphomas, clonal rearrangements were detected in skin samples in half of the cases. In the remaining patients, clonal TCR rearrangements could not be detected in the skin, but only in the blood and/or bone marrow specimens. Results from the analyses of sequential biopsies showed identical patterns of rearrangement in 11 patients. In the remaining patient, the pattern of rearrangement differed between skin and lymph node. These data confirm and extend previous reports and indicate that analysis of TCR β–chain genes by Southern blotting forms a useful supplement to other methods for the diagnosis of known and suspected CTCL. They also emphasize the importance of studying not only skin, but also extracutaneous sites.
    Type of Medium: Electronic Resource
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