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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 119 (1988), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Recombinant human IFN-γ, used for treatment of melanoma and renal carcinoma, was found to induce HLA-DR expression on human keratinocytes in vivo. HLA-DR antigens bound to keratinocytes of the basal and suprabasal layers of the epidermis were observed after intramuscular or intravenous injections of 0.5 mg/kg body weight IFN-y, 3 times a week. Keratinocyte-bound HLA-DR antigens were ftrst observed at the beginning of the third or fourth week of treatment, but HLA-DQ and HLA-DP antigens were never detected on keratinocytes. The intracytoplasmic constant (lO chain of the class II molecules was also not detectable within the keratinocytes. Paticnts who received IFN-α2 therapy, did not exhibit keratinocyte-bound HLA-DR antigens.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-2307
    Keywords: Langerhans cell histiocytosis ; HECA-452 ; Sialyl-Lex/sialyl-Lea ; Homing mechanisms ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The cutaneous lymphocyte associated antigen (CLA) recognized by the monoclonal antibody (moAb) HECA-452 plays a major role in the homing of lymphocyte subpopulations to the skin by binding to E-selectin on dermal microvessels. The factors responsible for the immigration of Langerhans cells (LC) and their precursors into the skin are still unknown, but because normal resting LC are also capable of expressing CLA, the antigen was proposed as a candidate LC-homing structure. To gain insight into these mechanisms, the expression of HECA-452 on neoplastic LC within and outside the skin was investigated in paraffin-embedded sections from 44 patients with localized and disseminated forms of Langerhans cell histiocytosis (LCH) presenting with proliferating cells positive for CD45, CD1a, S100 and HLADR. Irrespective of the clinical presentation or the type of organ involved, HECA-452-positive LC were detected in all biopsies tested (range 5-〉90%). The most prominent HECA-452 reactivity was observed in skin lesions and in areas with accumulations of eosinophilic granulocytes. Our data provide evidence for a heterogeneous expression of sLex/sLea structures in various stages of activated and/or differentiated LCH cells. Remarkably, CLA-antigen expression on LCH-cells was not restricted to cutaneous sites. In view of recent findings on the expression of HECA-452 on resting epidermal LC, our data are compatible with the view that local cytokine production by keratinocytes or cells from the surrounding infiltrate induce and/or modulate CLA expression on LC in both cutaneous and extra-cutaneous sites.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0584
    Keywords: Key words Related and unrelated donor BMT ; CML ; Long-term follow-up
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Between January 1983 and December 1997, 88 patients (36 female, 52 male, median age 37 years, range 19–57) with chronic myelogenous leukemia (CML) underwent allogeneic bone marrow transplantation (BMT) at the University Hospital of Vienna. Sixty patients were in chronic phase, 18 in accelerated phase, and ten in blast crisis. Marrow donors were HLA-identical siblings for 64 patients (BM 58, PBSC 6), 2-antigen-mismatched related donors (RD) for two, HLA-identical unrelated donors (URD) for 17, and 1-antigen-mismatched URD for five. The median time from diagnosis to BMT was 22 months (range 2–91), and 63 patients had received prior interferon (IFN)-alpha therapy, 46 (73%) for more than 6 months. Conditioning therapy consisted of cyclophosphamide (CY) and total body irradiation (TBI) in 71 patients and CY and busulfan (BU) in 16. One patient received etoposide and TBI. For graft-versus-host disease (GVHD) prophylaxis methotrexate (MTX) was given to 12 patients, MTX and cyclosporin A (CSA) to 67, CSA alone to four, and CSA and methylprednisolone to five. Durable engraftment was documented in 80 of 82 patients (98%). As of December 31, 1997, 52 patients (59%) were alive, 38 (58%) after sibling transplantation with a median observation time of 73 months and 14 (64%) after URD transplantation with a median observation time of 12 months. Probability of overall survival is 59%, for patients undergoing transplantation in chronic phase and 44% for patients undergoing transplantation in advanced stage CML. Probability of disease-free survival (DFS) after sibling and URD BMT is 55% and 59%, respectively. Ten patients (12%) experienced relapse of CML. Transplant-related mortality was 32% both after RD and after URD transplantation. Acute GVHD occurred in 53 of 80 evaluable patients (66%), consisting of grade III or IV in 14 patients (18%). Chronic GVHD developed in 40 of 63 eligible patients (63%), including extensive disease in 26 patients (41%). Thus, sibling and URD BMT offer high cure rates with acceptable toxicity to patients with CML.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0584
    Keywords: Severe aplastic anemia ; Bone marrow transplantation ; Graft versus host disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Survival and quality of life are reported in 23 pretransfused patients with severe aplastic anemia (SAA) who underwent bone marrow transplantation (BMT). The projected survival is 76% with 18 of 23 patients being alive 332 to 1677 days post graft (median: 842). 5 patients died between day 4 and 416. 12 of 17 patients at risk developed chronic graft versus host disease (GVH-D). 4 of these patients have a diminished quality of life due GVH-D related disabling manifestations. Autologous haemopoietic recovery was excluded in all patients by the demonstration of haemopoietic chimerism. We recommand age-adapted rejection prophylaxis; such strategy may help to diminish disabling graft versus host disease in otherwise haematologically reconstituted survivors.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Vernarbendes Pemphigoid ; Epiligrin-Autoantikörper ; Immunsuppressive Therapie ; Paraneoplasie ; Key words Cicatricial pemphigoid ; Anti-epiligrin autoantibodies ; Immunosuppressive therapy ; Paraneoplasia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A 71 year old female patient presented with erosions of the oral, genital and ocular mucosa, but without skin lesions. One year prior to the onset of lesions, a metastatic adenocarcinoma of the endometrium had been diagnosed. In perilesional skin, direct immunofluorescence showed linear deposits of C3 and IgG along the basement membrane zone. Indirect immunofluorescence demonstrated anti-basement membrane zone antibodies which bound exclusively to the dermal side of 1M NaCl-split skin. In immunoprecipitation studies, the autoantibodies bound a set of proteins characteristic of epiligrin/laminin-5, and they specifically immunoblotted the alpha-subunit of this protein. These results confirmed our presumed diagnosis of anti-epiligrin cicatricial pemphigoid. Combined treatment with methylprednisolone and dapsone resulted in prompt remission, despite progression of her malignancy. So far, only eight patients with anti-epiligrin cicatricial pemphigoid have been reported worldwide. Our patient represents the first case from Austria and is clinically and immunopathologically similar to previously reported cases.
    Notes: Zusammenfassung Berichtet wird über eine 71jährige Patientin mit erosiver Stomatitis, Vulvitis und Konjunktivitis, jedoch ohne Hautbeteiligung, bei der ein Jahr vor dem Auftreten der Schleimhautläsionen ein metastasierendes Adenokarzinom des Endometriums diagnostiziert worden war. Die Histologie zeigte eine junktionale Spaltbildung, die direkte Immunfluoreszenz periläsionaler Haut lineare Ablagerungen von C3 und IgG entlang der BMZ. Mit Hilfe der indirekten Immunfluoreszenz ließen sich niedrigtitrige anti-BMZ-Antikörper nachweisen, die in der Spalthautanalyse am Boden der artifiziellen Blase banden. Die Autoantikörper der Patientin präzipitierten ein Spektrum an Proteinen, das für Epiligrin/Laminin-5 typisch ist, und banden im Immunoblot spezifisch die alpha-Kette des Heteropeptids. Aufgrund dieser Ergebnisse stellten wir die Diagnose eines vernarbenden Pemphigoids vom anti-Epiligrin-Typ. Unter Behandlung mit Methylprednisolon und Dapson konnte eine rasche Remission erzielt werden, trotz Progression des metastasierenden Adenokarzinoms. Bisher wurden weltweit nur 8 Patienten mit vernarbendem Pemphigoid vom anti-Epiligrin-Typ beschrieben; unsere Patientin stellt den ersten in Österreich diagnostizierten Fall dar und gleicht sowohl klinisch als auch immunpathologisch den bisher beschriebenen Fällen.
    Type of Medium: Electronic Resource
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