| Objective: To summarize the clinical and laboratory characteristics,treatment and outcome of macrophage activation syndrome(MAS),and to provide experience for clinical diagnosis and treatment.Methods: The clinical data of 60 children with MAS in our hospital from December 2008 to December 2017 were collected and analyzed.The disease,clinical features,treatment methods of different hormones and the difference of prognosis were analyzed.Results:1.Among 60 children with MAS,primary diseases included 27 cases(45%)of systemic juvenile idiopathic arthritis(SJIA),15 cases of systemic lupus erythematosus(SLE),13 cases(21.67%)of Kawasaki disease(KD),4 cases of juvenile dermatomyositis(JDM)and 1 cases of mixed connective tissue disease(MTCD).2.Time for the occurrence of MAS: the median time of the occurrence of MAS is 29.5d,the earliest(median time 15d)is KD combined with MAS,and the latest is SLE with MAS(median time 199d).3.Cinical characteristics: all of the children showed fever(100%),of which 30 cases(50%)were accompanied by central nervous system symptoms such as headache,vomiting and convulsion,with different degrees of liver(75%),spleen(45%)and lymph node enlargement(43.33%).Laboratory tests showed a decline in three lines,among which hemoglobin was the most common(91.6%),followed by platelets(68.33%)and leukocyte decline(45%);and serological changes,including high level of alanine aminotransferase(75%),glutamic oxaloacetic transaminase(91.67%),triglyceride(83.33%)and serum ferritin(96.67%).52 cases were examined by bone marrow cytology,and 31 cases(59.61%)showed the phenomenon of phagocytosis of blood cells by bone marrow.4.MAS treatment and outcome: 55 cases were treated with glucocorticoid,of which 32 cases(58.18%)were treated with lower dose of hormone,23(41.82%)were treated with high dose of methylprednisolone(>15mg/kg·d),and 10 cases of glucocorticoid treatment were added with cyclosporin and etoposide.Compared with the lower does of methylprednisolone treatment group,the leukocyte decreased more significantly(P=0.04)and the lymphocyte count was lower(P=0.017).After 3 days of treatment,the platelet increase was more significant in the high dose methylprednisolone treatment group(P=0.04),and the decrease of alanine transaminase in this group was more obvious(P=0.048)after 5 days of treatment.There was no statistical difference in mortality between this two group.12 cases died(20%,4 cases gave up treatment),5 cases(8.3%)were lost to visit,and the remaining 43 cases(71.67%)improved after treatment.5.characteristics of death cases: compared with the cure group cases,the children with central nervous system symptoms,bleeding and respiratory failure were higher,the level of serum fibrinogen and the number of NK cells were lower(P=0.002,P=0.004),and the time of treatment in the death group was later than that in the cure group.(P=0.003).Conclusion: MAS is more common in SJIA,KD,SLE,JDM and other rheumatic diseases related MAS.Rheumatic disease has high fever,hepatosplenomegaly,blood drop,ferritin levels need to be wary of MAS.Bleeding,central nervous system symptoms,severe low fibrinogen,and multiple organ dysfunction may indicate poor prognosis.Glucocorticoids are still the first choice for the treatment of MAS,and the combination of cyclosporine and etoposide can improve the remission rate.Whether glucocorticoid pulse therapy is superior to medium or small dose hormone in the treatment of rheumatic MAS is still to be confirmed by large sample studies.Objective: To analyze the clinical and laboratory characteristics,treatment,and outcomes of Kawasaki Disease(KD)patients associated with macrophage activation syndrome(MAS)and to compare three diagnostic standards(the HLH 2009,SJIA-MAS 2005 and SJIA-MAS 2016).Methods: Retrospective review cases of MAS who has been treated and therapy in The Children’s Hospital of Chongqing Medical University,during 2007--2017.The clinical datas were analyzed.Results: There were 8 males and 4 females,with a median age of 25 months.The capital trigger of MAS was infection(8 cases,66.7%).Unabating high fever has been the initial manifestation for 12 p atients(100%),other conmen clinical features including hepatomegaly(11 cases,91.6%),splenomegaly(8 cases,66.7%)and lymphadenectasis(7 cases,58.3%).Further more,8 patients with different degree centra l nervous system symptoms.Laboratory examination showed a decrea se in hemoglobin(11 cases,91.6%),thrombocytopenia(8 cases,66.7%),and white blood cell decrease(4 cases,33.3%);serum transami nase(11 cases,91.6%),triglyceride(72.7%,8/11)and serum ferritin(100%,9/9)increased。11 patients(91.6%)had decreased ESR.Bone marrow cytology was performed in 10 cases,of which 8 cases(80%)showed hemophagocytic phenomenon.All the patients were diag nosed by SJIA-MAS(2005)criteria.All patients were treated with hi gh-dose intravenous immunoglobulin(IVIG)treatment,among which 3 cases were combined with methylprednisolone treatment,2 cases wi th more than two kings of immunosuppressive drugs(dexamethasone and ciclosporin or etoposide).Among the 12 patients,2 patients were lost to follow-up,4 cases,including two cases who withdrawn treatm ent,(33.3%)died due to hepatic encephalopathy,the remaining 6 cas es(50%)improved.Conclusion: Prolonged high fever as the first manifestation of MAS in Kawasaki disease.Hemogram and ESR decreased,elevated serum transaminase and ferritin may indicate MAS occurrence.High dose IVIG therapy for MAS in KD may effective.And,combined with glucocorticoid and immunosuppressive for therapy can improve the remission rate,severe central nervous system involvement may indicate terrible prognosis. |
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