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ABSTRACT: We report on the difficult differential diagnosis of liver involvement in disseminated Langerhans' cell histiocytosis (LCH). Three years after treatment of LCH involving the skull and pelvic bones, an 18-year-old girl presented with abdominal pain and cholestatic liver disease. At this time, liver biopsy showed portal infiltrates which were diagnosed as chronic non-suppurative destructive cholangitis. Two years later, she was icteric under progredient hepatic failure. A second liver biopsy revealed biliary fibrosis and granulomatous inflammation with destruction of the portal bile ducts. The morphological changes in both liver biopsies could be identified as LCH by immunohistochemical detection of CD1a and S-100-positive Langerhans' cells. Morphological changes and clinical findings in LCH of the liver may resemble primary sclerosing cholangitis or chronic non-suppurative destructive cholangitis. Therefore, LCH is an important differential diagnosis of chronic destructive cholangitis with cholestatic liver disease, especially in children and young adults. The diagnosis can be verified by S-100 and CD1a immunohistochemistry.
Der Pathologe 04/2003; 24(2):119-23. · 0.67 Impact Factor
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ABSTRACT: Zusammenfassung Berichtet wird ber die schwierige Differenzialdiagnose eines Leberbefalls im Rahmen einer Langerhans-Zell-Histiozytose (LCH). Bei einer 18-jhrigen Patientin waren 3 Jahre nach Chemotherapie eines eosinophilen Granuloms unklare Oberbauchbeschwerden und serologische Zeichen einer Cholestase aufgetreten. Histologisch fanden sich in der Leberbiopsie Vernderungen, die als chronische nichteitrige Cholangitis gedeutet wurden. Zwei Jahre spter bestand ein Ikterus mit massiven Leberfunktionsstrungen. Die erneute Biopsie zeigte eine fortschreitende Gallengangsdestruktion durch granulomartige histiozytenreiche Infiltrate sowie eine erhebliche bilire Fibrose. Die Vernderungen in beiden Biopsien konnten aufgrund der Expression von CD1a und S-100 einer LCH zugeordnet werden. Die LCH kann sich in der Leber in Form von Herdbefunden manifestieren oder klinisch und histologisch eine primr sklerosierende Cholangitis oder eine chronische nichteitrige Cholangitis vortuschen. Sie ist daher bei cholestatischen Gallenwegserkrankungen insbesondere im Kinder- und Jugendalter eine wichtige Differenzialdiagnose. Die Diagnose ist anhand der immunhistochemischen Identifizierung CD1a- und S-100-exprimierender Langerhans-Zell-Infiltrate mglich. Abstract We report on the difficult differential diagnosis of liver involvement in disseminated Langerhans' cell histiocytosis (LCH). Three years after treatment of LCH involving the skull and pelvic bones, an 18-year-old girl presented with abdominal pain and cholestatic liver disease. At this time, liver biopsy showed portal infiltrates which were diagnosed as chronic non-suppurative destructive cholangitis. Two years later, she was icteric under progredient hepatic failure. A second liver biopsy revealed biliary fibrosis and granulomatous inflammation with destruction of the portal bile ducts. The morphological changes in both liver biopsies could be identified as LCH by immunohistochemical detection of CD1a and S-100-positive Langerhans' cells. Morphological changes and clinical findings in LCH of the liver may resemble primary sclerosing cholangitis or chronic non-suppurative destructive cholangitis. Therefore, LCH is an important differential diagnosis of chronic destructive cholangitis with cholestatic liver disease, especially in children and young adults. The diagnosis can be verified by S-100 and CD1a immunohistochemistry.
Der Pathologe 02/2003; 24(2):119-123. · 0.67 Impact Factor
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ABSTRACT: To evaluate safety and efficacy of the protease inhibitor combination ritonavir/indinavir 100/800 mg twice daily plus 2-3 nucleoside reverse transcriptase inhibitors (NRTI) in antiretroviral-naive patients.
Within this open-label, uncontrolled multicentre trial, antiretroviral-naive patients (n = 57) with median baseline HIV-RNA of 308,000 copies/mL (range 170-3.01 million copies/mL) and median CD4 cell count of 50 cells/microL (range 0-853 cells/microL) were started on 2-3 NRTIs plus ritonavir/indinavir 100/800 mg twice daily. CD4 cell counts and HIV-RNA were determined at weeks 0, 4, 8, 12, 16, 20, 24 and 48. Statistical analysis was performed on treatment as well as intent-to-treat.
Viral load decreased by a median of 3.79 log10 copies/mL (range 2.0-4.60 log10 copies/mL) until week 48. At week 48, 23/57 (40%, intent-to-treat) patients showed a viral load </= 80 copies/mL. In parallel, median CD4 cell counts increased by a median of 149 cells/microL (range -60-420 cells/microL). Median triglycerides and cholesterol increased from baseline 160 mg/dL (range 33-364 mg/dL) to 218 mg/dL (range 110-527 mg/dL) at week 48 and from 166 mg/dL (range 63-262 mg/dL) to 233 mg/dL (range 95-359 mg/dL), respectively. Twenty-seven of fifty-seven patients (47%) discontinued study medication, 19 (33%) due to nephrolithiasis. Two patients changed their antiretroviral regimen after failing virologically.
Ritonavir/indinavir 100/800 mg twice daily appears to be effective up to 48 weeks despite high baseline viraemia and low CD4 cell count in antiretroviral-naive patients. However, discontinuation due to adverse events, especially nephrotoxicity, is frequent and limits treatment duration. Therefore, extra hydration appears inevitable with this combination.
HIV Medicine 10/2002; 3(4):277-82. · 3.01 Impact Factor
