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

作　　者：Sarah Georgiadou Nikolaos K Gatselis Aggelos Stefos Kalliopi Zachou Konstantinos Makaritsis Eirini I Rigopoulou George N Dalekos 

机构地区：[1]Department of Medicine and Research Laboratory of Internal Medicine,University Hospital of Larissa,Larissa 41110,Greece [2]Institute of Internal Medicine and Hepatology,University Hospital of Larissa,Larissa 41447,Greece

出　　处：《World Journal of Clinical Cases》2019年第21期3394-3406,共13页世界临床病例杂志

摘　　要：BACKGROUND Secondary haemophagocytic lymphohistiocytosis(sHLH)is a rare lifethreatening condition mainly associated with underlying infections,malignancies,and autoimmune or immune-mediated diseases.AIM To analyse all sHLH cases that were diagnosed and managed under real-world circumstances in our department focusing on the treatment schedule and the outcome.METHODS 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 rate.Most 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 prednisolone.RESULTS 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 them.Underlying 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 steroids.Overall,sHLH resolved in 76%of patients,15%died within the first month but 82.5%of patients were still alive 6 mo after diagnosis.Univariate analysis showed older age,anaemia,throBACKGROUND Secondary haemophagocytic lymphohistiocytosis(sHLH) is a rare lifethreatening condition mainly associated with underlying infections,malignancies, and autoimmune or immune-mediated diseases.AIM To analyse all sHLH cases that were diagnosed and managed under real-world circumstances in our department focusing on the treatment schedule and the outcome.METHODS 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 rate.Most of patients had received after consent intravenous γ-immunoglobulin(IVIG) for 5 d(total dose 2 g/kg) in combination with intravenous steroid pulses followed by gradual tapering of prednisolone.RESULTS 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 them.Underlying 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 in2/80(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 steroids.Overall, sHLH resolved in 76% of patients, 15% died within the first month but 82.5% of patients were still alive 6 mo after diagnosis.Uni

关 键 词：Haemophagocytic syndrome Haemophagocytic LYMPHOHISTIOCYTOSIS γ-immunoglobulin LEISHMANIASIS FERRITIN 

分 类 号：R73[医药卫生—肿瘤]