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Efficient management of secondary haemophagocytic lymphohistiocytosis with intravenous steroids and γ-immunoglobulin infusions

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World Journal of Clinical Cases 2019年第21期7卷 3394-3406页

作者： Sarah Georgiadou Nikolaos K Gatselis aggelos stefos Kalliopi Zachou Konstantinos Makaritsis Eirini I Rigopoulou George N Dalekos Department of Medicine and Research Laboratory of Internal Medicine University Hospital of LarissaLarissa 41110Greece Institute of Internal Medicine and Hepatology University Hospital of LarissaLarissa 41447Greece

BACKGROUND Secondary haemophagocytic lymphohistiocytosis(sHLH)is a rare lifethreatening condition mainly associated with underlying infections,malignancies,and autoimmune or immune-mediated *** To analyse all sHLH cases that were diagnosed and managed under real-world circumstances in our department focusing on the treatment schedule and the *** Prospectively collected data from all adult patients fulfilling the criteria of sHLH who diagnosed and managed from January 1,2010 to June 1,2018,in our department of the tertiary care university hospital of Larissa,Greece,were analysed retrospectively(n=80;52%male;median age:55 years).The electronic records and/or written charts of the patients were reviewed for the demographic characteristics,clinical manifestations,underlying causes of sHLH,laboratory parameters,treatment schedule and 30-d-mortality *** of patients had received after consent intravenousγ-immunoglobulin(IVIG)for 5 d(total dose 2 g/kg)in combination with intravenous steroid pulses followed by gradual tapering of *** Seventy-five patients(94%)reported fever>38.5°C,47(59%)had liver or spleen enlargement and 76(95%)had ferritin>500 ng/mL including 20(25%)having considerably high levels(>10000 ng/mL).Anaemia and thrombocytopenia occurred in 72%and leucopoenia in 47%of *** infections were diagnosed in 59 patients(74%)as follows:leishmaniasis alone in 15/80(18.9%),leishmaniasis concurrently with Coxiella Burnetti or non-Hodgkin lymphoma in 2/80(2.5%),bacterial infections in 14/80(17.5%)including one case with concurrent non-Hodgkin lymphoma,viral infections in 13/80(16.3%),fungal infections in 2/80(2.5%),infections by mycobacteria in 1/80(1.3%)and unidentified pathogens in 12/80(15%).Seventy-two patients(90%)had received combination treatment with IVIG and intravenous ***,sHLH resolved in 76%of patients,15%died within the first month but 82.5%of patients were still alive 6 mo after ***

关键词： Haemophagocytic syndrome Haemophagocytic lymphohistiocytosis γ-immunoglobulin Leishmaniasis Ferritin

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Reversion of severe hepatopulmonary syndrome in a non cirrhotic patient after corticosteroid treatment for granulomatous hepatitis:A case report and review of the literature

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World Journal of Gastroenterology 2006年第2期12卷 336-339页

作者： Nikolaos Tzovaras aggelos stefos Sarah P Georgiadou Nikolaos Gatselis Georgia Papadamou Eirini Rigopoulou Maria Ioannou Ioannis Skoularigis Georgios N Dalekos Department of Medicine Academic Liver UnitMedical SchoolUniversity of Thessaly41222 LarissaGreece Department of Pathology Medical SchoolUniversity of Thessaly41222 LarissaGreece Division of Cardiology Medical SchoolUniversity of Thessaly41222 LarissaGreece

Hepatopulmonary syndrome （HPS） is defined as a clinical triad including liver disease, abnormal pulmonary gas exchange and evidence of intrapulmonary vascular dilatations. We report a 61-year-old male presented with fatigue, long-lasting fever, loss of weight, signs of portal hypertension, hepatosplenomegaly, cholestasis and progressive dyspnoea over the last year. Clinical, laboratory and histological findings confirmed the diagnosis of granulomatous hepatitis. HPS due to hepatic granulomainduced portal hypertension was proved to be the cause of severe hypoxemia of the patient as confirmed by contrast-enhanced echocardiography. Reversion of HPS after corticosteroid therapy was confirmed by a new contrastenhanced echocardiography along with the normalization of cholestatic enzymes and improvement of the patient＇ s conditions. This is the first case of complete reversion of HPS in a non-cirrhotic patient with hepatic granuloma, indicating that intrapulmonary shunt in liver diseases is a functional phenomenon and HPS can be developed even in miscellaneous liver involvement as in this case.

关键词： Hepatopulmonary syndrome Granulomatous hepatitis Liver cirrhosis Orthodeoxia Platypnoea

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Hepatitis B virus reactivation in hepatitis B virus surface antigen negative patients receiving immunosuppression: A hidden threat

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World Journal of Hepatology 2013年第7期5卷 387-392页

作者： Kalliopi Zachou Alexandros Sarantopoulos Nikolaos K Gatselis Themistoklis Vassiliadis Stella Gabeta aggelos stefos Asterios Saitis Panagiota Boura George N Dalekos Department of Medicine and Research Laboratory of Internal Medicine School of Medicine University of Thessaly Clinical Immunology Unit 2nd Department of Internal Medicine Hippokration General Hospital Aristotle University of Thessaloniki First Medical Propedeutic Department Medical School Aristotle University of Thessaloniki AHEPA Hospital Thessaloniki

AIM: To present the characteristics and the course of a series of anti- hepatitis B virus core antibody (HBc) antibody positive patients, who experienced hepatitis B virus (HBV) reactivation after immunosuppression. METHODS: We retrospectively evaluated in our tertiary centers the medical records of hepatitis B virus surface antigen (HBsAg) negative patients who suffered from HBV reactivation after chemotherapy or immunosuppression during a 3-year period (2009-2011). Accordingly, the clinical, laboratory and virological characteristics of 10 anti-HBc (+) anti-HBs (-)/HBsAg (-) and 4 anti-HBc (+)/antiHBs (+)/HBsAg (-) patients, who developed HBV reactivation after the initiation of chemotherapy or immunosuppressive treatment were analyzed. Quantitative determination of HBV DNA during reactivation was performed in all cases by a quantitative real time polymerase chain reaction kit (COBAS Taqman HBV Test; cut-off of detection: 6 IU/mL). RESULTS: Twelve out of 14 patients were males; median age 74.5 years. In 71.4% of them the primary diagnosis was hematologic malignancy; 78.6% had received rituximab (R) as part of the immunosuppressive regimen. The median time from last chemotherapy schedule till HBV reactivation for 10 out of 11 patients who received R was 3 (range 2-17) mo. Three patients (21.4%) deteriorated, manifesting ascites and hepatic encephalopathy and 2 (14.3%) of them died due to liver failure. CONCLUSION: HBsAg-negative anti-HBc antibody positive patients can develop HBV reactivation even 2 years after stopping immunosuppression, whereas prompt antiviral treatment on diagnosis of reactivation can be lifesaving.

关键词： Immunosuppression Hepatitis B Antihepatitis B virus core antibody positivity Occult hepatitis B virus infection Rituximab

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