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 

作者机构：Department of Medicine and Research Laboratory of Internal MedicineUniversity Hospital of LarissaLarissa 41110Greece Institute of Internal Medicine and HepatologyUniversity Hospital of LarissaLarissa 41447Greece 

出 版 物：《World Journal of Clinical Cases》 (世界临床病例杂志)

年 卷 期：2019年第7卷第21期

页      面：3394-3406页

核心收录：

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主　　题：Haemophagocytic syndrome Haemophagocytic lymphohistiocytosis γ-immunoglobulin Leishmaniasis Ferritin 

摘      要：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 fever38.5°C,47(59%)had liver or spleen enlargement and 76(95%)had ferritin500 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 ***