| Objective:To summarize the clinical features of secondary Hemophagocytic syndrome(HPS)associated with Talaromycosis marneffei(TSM),raise the clinicians’awareness,and decrease the misdiagnosis and missed diagnosis.Methods:This retrospective study was conducted between August 2012 and February 2019 at multiple research centers.HIV-negative patients(n=126)who presented with culture and/or histopathological proof of TSM were enrolled.Of nine patients with Talaromycosis-associated secondary HPS,then the clinical presentations,treatment and prognosis were analyzed.Results:Seven male and two female,median age:2 years[range:1-41years]).5 patients were younger than 3 years old.The median time from the onset of symptoms to diagnosis was 1.8 months(range:0.9-6.5 months).One child had congenital megacolon,the other 5 children had premature or recurrent infections.Two adults had hyperthyroidism or systemic lupus erythematosus.Fever and anemia could be found in all patients.Seven patients had cough,six had weight loss,and two each had oral thrush,lymphadenopathy,hepatomegaly,and splenomegaly.One patient had digestive symptoms and joint pain,and dyspnea was also present.All patients showed reduced hemoglobin concentrations,the median were 77.0 g/L(range:66.1-81.8)g/L,thrombocytopenia,the median were 25.4×10~9/L(range:16.6-82.8)×10~9/L,and hypoproteinemia.Six patients(66.7%)showed liver dysfunction,hypofibrinogenemia,and prothrombin time prolonged,respectively.Hypertriglyceridemia in 4 patients.All the patients showed hyperseroferritinemia,elevated lactate dehydrogenase,D-dime,C-reactive protein concentrations,erythrocyte sedimentation rates and procalcitonin.Four showed reduced globulin,serum Immunoglobulin G levels were increased in three patients(37.5%),and 6 of 7 patients(85.7%)showed low NK cell counts.Chest videography indicated that all patients had different pulmonary lesions,four(44.4%)had diffused patchy density shadow.Seven(77.8%)had pleural inflammatory reaction and/or pleural effusion,and six(66.7%)had mediastinal and/or hilar lymphadenopathy.Two(22.2%)had pericardial effusion,cavities,pulmonary consolidation,and osteolytic lesions in the ribs.T.M was isolated from venous blood(6/9,66.7%),bone marrow(3/5,60%),sputum samples(2/5,40%),and dermal lesion secretions(2/2,100%).In addition,three cases were diagnosed with T.M infection by histopathology or cytology of specimens obtained from bone marrow(2/7,28.6%)or lymph nodes(1/3,33.3%).Bone marrow aspirate from three patients(3/7,42.9%)showed histiocytic hyperplasia and marked hemophagocytosis.Patients who were diagnosed with TSM-associated secondary HPS satisfied the diagnostic criteria of HLH-2004.One patient did not receive antifungal therapy and died.Eight received antifungal therapy,6 patients received voriconazole,2 patients received intravenous fluconazole.Eight of nine patients received a specific treatment for HPS after diagnosis,treatment included corticosteroids(CS)and/or intravenous immunoglobulin(IVIG)in six patients,CS+etoposide+IVIG in two patients.One patient without anti-HLH treatment.Four died during treatment,3improved,and 1 lost to follow up.Conclusions:(1)T.M infection leading to Hemophagocytic syndrome is extremely rare,more common in children with immunodeficiency and adults with underlying disease.(2)In T.M infection endemic areas,when patients are diagnosed with HPS,after excluding viral infections and other types of infections,infection caused by T.M should also be excluding.(3)T.M fungemia associated with HPS was related to high mortality,once diagnosed,timely and effective antifungal treatments and supportive care are essential;if antifungal treatment fails,individual evaluation should be used to determine whether hormone therapy or systemic chemotherapy is needed. |
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